key: cord-034340-3ksfpaf7
authors: nan
title: Proceedings of the 26th European Paediatric Rheumatology Congress: part 2: Virtual. 23 - 26 September 2020
date: 2020-10-28
journal: Pediatr Rheumatol Online J
DOI: 10.1186/s12969-020-00470-5
sha: 
doc_id: 34340
cord_uid: 3ksfpaf7

nan

Introduction: Juvenile idiopathic arthritis (JIA) represents the most common pediatric chronic rheumatic disease. Children with JIA present an increased risk of infections, due to the immune-regulatory effects of disease modifying antirheumatic drugs (DMARDs); many of these infections are vaccine-preventable. Nevertheless, suboptimal vaccinations rates are reported in children with JIA. Objectives: To evaluate vaccination coverage in a population of children with JIA and to describe the prevalence of the adverse events following immunization (AEFIs) in our cohort. Methods: A single-centre retrospective study was conducted by reviewing medical records of all JIA patients, diagnosed according to ILAR criteria, admitted to the Pediatric Rheumatology Unit of University of Naples Federico II from January to December 2019. Parents were asked to provide the vaccinations records in form of the vaccination booklet. The occurrence of AEFIs was explored by telephone interviews.

Introduction: Intrarticular corticosteroid injections (IACI) are widely used in the management of patients with juvenile idiopathic arthritis (JIA). General anesthesia can be avoided in case of a small number of joints to inject or in older children. However, pain and anxiety may reduce the patient compliance to IACI, and may compromise the accuracy of the procedure. In order to overcame such problems, the use of appropriate methods of pain and anxiety control is advisable. Objectives: To assess the effectiveness and satisfaction of patients undergoing IACI with the use of topical numbing agent or under minimal sedation. Methods: Patients with JIA who underwent an IACI of up to 3 joints were recruited. Depending on age and number of joints to treat, a group of patients (group A) were injected with the application 30 minutes prior the procedure of a topical numbing agent (prilocaine+lidocaine) to the skin over the injection site. Another group of patients (group B) were treated under minimal sedation (Ketorolac/Tramadol or Morphine + Midazolam). The physician was asked to record the degree of motion and pain of the patient during the procedure and the patient (or parents for patients aged less than 4 years) was asked to report the degree of pain and satisfaction on a Visual Analogue Scale (VAS) from 0 to 10. Results: Twenty-seven patients were enrolled for a total of 30 procedures, 17 and 13 of them in group A and B, respectively. The median age at the procedure was 10 years for group A and 11 years for group B. For group A median pain scores for patients, parents and physicians were 2, 2 and 1.5, respectively. In patients of group B who underwent the IACI under Ketorolac/Tramadol the median pain scores for patients, parents and physicians were 3, 5.25 and 2.5, whereas in patients treated with Morphine median pain scores were 6, 6 and 2, respectively. Overall, we found that pain as reported by the patient/parent were higher with increase in the number of sites injected (and, consequently, duration of procedure) and age of patient. Amount of motion during procedures was overall negligible. The majority of patients/parents was satisfied for the procedures. Only 2 patients treated with Midazolam had psychomotor agitation during the IACI. Conclusion: IACI in a small number of sites without the use of general anesthesia is well tolerated by patients. The level of pain perceived from patients is irrespective of the power of the painkiller used, but seems to correlate with the duration of the procedures. It is possible that, in the paediatric age, the psychoemotional component seems to be decisive, with a progressive loss of tolerance with the increase in the number of injected joints.

for GC-MS analysis of the steroid hormone metabolites age and sexmatched healthy controls were matched to each patient. Patients were excluded if they were treated with corticosteroids in the preceding 3 months. Results: Of the 35 metabolites measured, 23 were significantly lower in JIA patients before the Etanercept treatment compared to the healthy control group. One day after the injection only 5 metabolites were still significantly lower in the JIA patients and all the other 30 metabolites normalized and were similar to the control group. Urine metabolite ratios reflecting CYP21 and 11β-HSD2 enzymatic activity indicate that these two enzyme activities were lower in JIA patients. The slowest recoveries noted were for metabolites of DHEAS and 17 OH pregnenolone. Conclusion: Prior to Etanercept treatment almost all urine adrenal metabolites were significantly lower mainly due to the active inflammatory process. Immediately after the treatment many metabolites raised to normal values as in the control group. The two adrenal enzymes that were found to be affected in JIR are CYP21 and 11β-HSD2. Blocking TN alpha immediately restore adrenal function in JIA.

Introduction: Patients with juvenile idiopathic arthritis (JIA) receive adalimumab treatment. Adalimumab is a monoclonal antibody that blocks TNF-α and is structurally and functionally similar to human IgG 1 . Nevertheless, there are reports of the development of anti-drug antibodies. The production of these antibodies may be associated with treatment failures (a decrease in the effectiveness of therapy or drug inefficiency that developed over time) and hypersensitivity reactions. To our knowledge, there is currently limited information on the availability of adalimumab antibodies (AAA) in patients with JIA. Objectives: to evaluate the prevalence rate and the clinical significance of AAA in patients with JIA on adalimumab treatment. Methods: 26 patients with JIA were examined, 17 of whom had the oligoarticular form of the disease, 7 of them with uveitis, and 9 patients had the polyarticular form of the disease, 3 of them with uveitis. Among them, there were 13 (50%) girls and 13 (50%) boys. The mean age was 11.0 ± 3.4 years; the mean disease duration was 4.1 ± 2.2 years. Patients received adalimumab (at least 1 year before the study) with concomitant administration of methotrexate (MTX) or adalimumab only -13 children who did not receive MTX for at least 3 months prior to the study as a result of either adverse events of MTX administration (5 patients) or permanent drug remission (8 patients). Before starting adalimumab therapy, all participants were treated with MTX. The mean duration of adalimumab treatment for these patients was 1.8 ± 1.0 years. The serum AAA level of antibodies was determined using the enzyme immunoassay (EIA) method. This method determines both free and bound antibodies to adalimumab at reference values less than 10 AU/ml. a and was used every 2 weeks for 3 months. The values were presented as mean ± standard deviation. Data processing and analysis were carried out using Pearson's chi-squared test and Spearman's correlation test. Results: 8 (31%) of the 26 patients enrolled in the study had AAApositive results. The mean AAA level in positive patients was 40.8 ± 20.1 AU/ml. Further disease relapses tended to occur significantly more often in AAA-positive patients than in AAA-negative ones (χ2 = 5.46, p = 0.019). Thus, 5 of 8 (62.5%) AAA-positive children had at least 1 exacerbation of the disease within 3 months, compared with 3 of 18 (16.7%) in AAA-negative ones. 7 out of 8 (87.5%) AAApositives did not take MTX for at least 3 months compared to 6 out of 18 (33.3%) in AAA-negative ones. Thus, AAAs are found to be significantly more frequent without concomitant administration of MTX in the treatment of JIA (χ2 = 6.5, p = 0.01). There were no observed adverse events or side effects during adalimumab therapy. No significant correlation was found between the presence of AAA and sex, Introduction: Advances on molecular medicine, illumination of the cytokine network and the immune pathways shed light on the etiopathogenesis for a better understanding of Juvenile idiopathic arthritis (JIA). However, the fact that the course of the disease differs individually strongly suggests the effect of external factors. Objectives: The current study was undertaken to evaluate sociodemographic and sociocultural features, parent behavior, the gestation and breastfeeding period, nutritional status of early childhood in our patients with JIA, and to determine their relationship with disease activity, damage index, remission time, and relapse rate. Methods: The study was conducted with a face-to-face questionnaire method with the parents of 171 patients with JIA and 183 healthy children. The medical patient records were reviewed. Juvenile Arthritis Disease Activity Score (JADAS) 27, Wallace clinical inactive disease criteria, Juvenile Arthritis Damage Index (JADI), and relapse rates were used to assess the general medical condition of each patient. Results: The median age of JIA patients (n = 171) was 13 (3) (4) (5) (6) (7) (8) (9) (10) (11) (12) (13) (14) (15) (16) (17) (18) (19) (20) , with a female ratio of 59,1%. Age at disease onset was 7 (1) (2) (3) (4) (5) (6) (7) (8) (9) (10) (11) (12) (13) (14) (15) (16) years. The first remission time was 5(1-17) months. The patients were evaluated according to disease subtypes and treatment modalities. There was no difference in the duration of breastfeeding according to the distribution of the subtypes (p = 0,97). When the breastfed and formula-fed patients were compared, there was a marginally significant difference in terms of first remission time (p = 0,05), whereas there was a significant difference in relapse rate in patients who introduced to cow milk early (<12 months) (p = 0,019). The early risk factors and their relationship with the disease are presented in Table 1 . Both breastfeeding durations and maternal literacy levels showed a significant difference in terms of relapse rates (p = 0,01; p=0,03, respectively). There was no significant difference in breastfeeding durations and gestational risks between the patients and the healthy group (p = 0,1; p= 0,65), respectively. However, the smoking rate among family members was significantly higher in the patient group (p = 0,03). Conclusion: In patients with juvenile idiopathic arthritis, breastfeeding rate and duration did not differ when compared to healthy controls. However, breastfeeding duration, cow's milk commence age, and maternal literacy appeared to be relevant to the relapse rates. Going to preschool both influence the remission time and relapse rate. These findings suggest a role for parental attitude and nutritional status during early childhood in the course of JIA.

None declared Introduction: Immunogenicity and low trough concentrations have been associated with adalimumab treatment failure in several studies of paediatric inflammatory diseases, indicating the possible value of therapeutic drug monitoring (TDM). Adalimumab efficacy may be improved by changing dose or treatment intervals based on drug concentrations. However, lack of standardization, assay heterogeneity, and paucity of research hinder the implementation of TDM in clinical practice.

Objectives: To assess the relationship of trough concentrations, immunogenicity and adalimumab response in paediatric patients with JIA. Methods: Monocentric cohort study of patients ≤18 years with JIA treated with adalimumab due to active arthritis. Clinical data and plasma samples were collected during routine follow-up. Adalimumab trough concentrations were measured by liquid chromatographytandem mass spectrometry (LC-MS/MS). Anti-adalimumab antibodies were measured in samples with trough concentrations <5mg/l. Disease activity was evaluated using the clinical Juvenile Arthritis Disease Activity Score with 71 joint count (cJADAS71). Response to adalimumab was defined as at least 50% reduction of disease activity within 3 months of therapy followed by clinical inactive disease or minimal disease activity after 6 months. The latter was defined as cJADAS71 ≤1.5 and ≤2.5, for oligoarthritis and polyarthritis, respectively, or an active joint count equal to zero when cJADAS71 was unavailable.

Results: 36 adalimumab trough samples were available from 35 JIA patients. Although there was no significant difference in median adalimumab dose, trough concentrations were significantly lower in patients with secondary failure compared to primary failure or an adequate adalimumab response (p-values <0.01). In addition, there were 11 samples with trough concentrations <5mg/l, 9 in the group with secondary failure and 2 in the group with adequate adalimumab response (Table 1) . Conclusion: Adalimumab trough concentrations were significantly lower in JIA patients with secondary failure compared to primary failure or an adequate response to adalimumab. Anti-adalimumab antibodies were present in 8 out of 11 samples with trough concentrations <5mg/l. Adalimumab trough concentration measurements may identify JIA patients that would benefit from increased doses or shorter treatment intervals. In addition, JIA patients with primary failure and adequate adalimumab trough concentrations may respond better to biologic agents with other therapeutic targets. Although biologic agents have improved disease outcome of patients with JIA, concentration measurements using reliable and cost-effective methods, such as LC-MS/MS, could further improve efficacy of biologic agents and guide treat-to-target strategies. Introduction: Most studies of physical fitness (PF) in juvenile idiopathic arthritis (JIA) have shown decreased levels of maximal oxygen consumption (VO 2 max) compared to healthy peers. In JIA, boys have higher PF-levels than girls and younger children have higher levels than adolescents, congruently with data of healthy peers. Previously, we have shown that more than half of JIA-patients had below normative average of VO 2 max. However, monitoring physical activity (PA) using accelerometry, 68% of boys and 39% of girls with JIA fulfilled the recommendations of WHO of ≥1 hour of PA per day, which was comparable to normative values (61%/39%). Moreover, patients reporting engagement more than 7 hours per week in club-sports exceeded accelerometry values of healthy peers. Objectives: To explore the association between PF and specific sport habits in 10 to 16-year-old JIA-patients, related to gender, BMI, disease activity, and pain, and comparing the most fit quartile (Q4) of patients (respectively boys and girls) to the least fit quartile (Q1). Methods: Sixty patients with JIA performed an indirect ergometertest of VO 2 max (Watt max test) and answered the Physical Activity and Sport Questionnaire (PASQ). Objective PA-monitoring for one week was conducted using the GT1M accelerometer. Cut-offs for moderate-high and high intensity PA were set to >1000 and >2500 counts per minute, respectively. Disease activity was assessed with the JADAS-27, current pain and worst pain last week were measured on visual analogue scales (VAS) and in a one-week pain diary using the Faces Pain Scale-Revised (FPS-R).

Results: Girls with JIA (n=36) had lower mean PF than the boys (n= 24) (36.5±8.2/43.4±6.73 ml/kg/min), being below normative values, respectively. In both genders the most fit boys and girls (Q4; 49.3-57/ 40.9-54) had average to well-above normative average PF. The least fit boys (Q1; 33.5-37.4) all had PF-levels well-below normative average. In girls Q1-levels (18.7-30.9) were well-below to below normative average. We found significant differences between most fit (Q4) and least fit (Q1) patients regarding patient´s global wellbeing (p=0.040) and pain diary (p=0.026). These differences were not significant when separating genders, though differences were more pronounced in girls. The least fit girls (Q1) had significantly higher disease activity (JADAS-27) than the most fit girls (Q4)(p=0.019). The most fit boys and girls (Q4) engaged equally in high intensity sports (soccer: 3/24; 2/36, handball: 0/24; 2/36, gymnastics: 2/24; 4/ 36, rowing: 1/24; 0/36). However, more boys than girls played soccer (11/24; 3/36), whereas more girls preferred sports of lower intensity (riding: 8/36; 0/24). Eight of 11 boys in soccer and 2 boys in gymnastics or rowing had below average to well-above normative average of PF (Q3+Q4: 41.6-57). Three girls in gymnastics, 2 girls in soccer, and 2 girls in handball were in Q4 (40.9-54) with levels of average to well-above average PF. The third girl in soccer was in Q2 (31-36.3) with levels of well-below to below normative average. None of the riding girls were in Q4 and only 1 was in Q3 (36.3-40.8) (Below to average normative PF). Comparing accelerometer-monitored values of PA-intensity in girls with low (Q1) and high (Q4) PF, PAvalues of Q1 were significantly lower than in Q4. The same tendency was observed in boys, but not to significance. Conclusion: Our results are in accordance with most other studies of PF in JIA, adding to the knowledge of gender-specific differences in PF and type and behavior in sport activities. It emphasizes the need of regular PF-testing and guidance in high intensity PA and sport in order to improve PF and avoid the risks of inactivity and lifestyle diseases in JIA.

pg/ml), which also showed the highest the frequency of detection of its increase. It was absent in sJIA (7.52±4.74 pg/ml). The highest values of IL-17R (1849836.4±176751 pg/ml) were in the middle age group. The data obtained suggest the compensatory value of increasing IL-17R and the simultaneous initiation of inflammatory and anti-inflammatory processes during exacerbation of JIA. Assessment of the ratios of stimulating and inhibiting cytokines showed in patients with uveitis, the ratio of IFN-γ/IL-1β (4379.29 ±476.83) was higher than with other JIA (from 60.84 ± 14.92 in oJIA to 105.20± 66.01 in pJIA) and IFN-γ/IL-17R (4474.01 ±3899.19 versus from 20.14± 11.48 in oJIA to 934.55±931.37 in sJIA). An increase of IL-1β/IL-17R ratio was characteristic only for sJIA (34.12±26.17). All of these ratios increased with disease activity (r=0.22-0.37) & they did not reflect the unpleasant course of the disease. Methods: In this multicenter, case-control study, 113 fecal samples were collected from 91 children with JIA, with 72 of these samples collected from untreated children (67 of whom were treatment-naïve children). Samples from 28 children with JIA were collected during treatment with MTX as single treatment and samples from 13 children during treatment with ETN. Of those 13 children, four were treated with ETN as single treatment and nine had a combination of ETN and MTX. We compared 28 children on single treatment with MTX with 57 untreated children (52 treatment-naïve), and 13 children on treatment with ETN (nine in combination with MTX) with 62 untreated children (57 treatment-naïve). We also did pairwise comparisons of samples taken before any medication was given (n = 22) and samples taken during ongoing treatment with MTX (n = 14) or ETN (n = 8, four in combination with MTX). The microbiota was characterized by sequencing amplicons from the V3 and V4 regions of the 16S rRNA gene. Alpha diversity of the fecal samples was measured using the Chao-1 index and the Shannon diversity index. To compare these indices between treated children and untreated children, we used a logistic regression model with age at sampling as a covariate. For pairwise analyses, we used the Wilcoxon signed-rank test.

To analyze the community composition of the microbiota, principal coordinate analysis (PCoA) plots based on Bray-Curtis distances were generated for visual comparisons, and analysis of similarity (ANOSIM) was used to test for differences.

Analyses for relative abundances of taxa were performed at three taxonomic levels (phyla, families, and genera), and logistic regression with age as a covariate was used for calculations of differences between treated and untreated children, while the Wilcoxon signedrank test was used for pairwise comparisons. Significance was set to p < 0.05 and corrections for multiple comparisons were made using the Benjamini-Hochberg method.

Results: Analyses showed no significant differences in α-diversity between children treated with MTX or ETN and untreated children, and pairwise comparisons of samples before and during treatment with MTX or ETN also showed no differences. PCoA plots for children treated with MTX or ETN, in comparison with untreated children, did not show any clustering. ANOSIM showed no significant differences between treated and untreated children. PCoA plots were also made for the pairwise comparisons of children sampled before and during treatment, and according to that analysis the community compositions of microbiota did not change in any uniform way during treatment with either MTX or ETN. Furthermor, analyses of relative abundances of specific taxa did not reveal any significant results in any of the comparisons, after adjustment for multiple analyses. Conclusion: Treatment with MTX or ETN did not alter the composition of fecal microbiota in children with JIA.

Introduction: Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood and an important cause of shortterm and long-term disability if patients are not treated appropriately. By definition, JIA clinically presents with peripheral joint inflammation of unknown origin, persisting for at least six consecutive weeks and starting before the age of 16 years. The predominant subtypes, i.e. oligoarticular (oligo) and polyarticular (poly) JIA, have long been assumed autoimmune diseases caused by dysregulation of the adaptive immune system, with a central role for autoreactive T cells belonging to the Th1 and Th17 lineages and autoantigens that may include aggrecan, fibrillin, matrix metalloproteinase (MMP)-3 and heat shock proteins. Nevertheless, the original T cell-centered hypothesis has been challenged since it does not cover nor completely explain the full spectrum of immune-pathological phenomena observed in patients. lien.desomer@uzleuven.b Objectives: Emerging evidence suggests a potentially important role for neutrophils in JIA pathogenesis. Here, we investigated extensively the phenotypical features of neutrophils present in the peripheral blood and inflamed joints of JIA patients. Methods: Synovial fluids and parallel blood samples from patients with oligo-or polyJIA and blood samples from healthy children were collected. Multicolor flow cytometry panels allowed for in-depth phenotypical analysis of neutrophils, focusing on the surface expression of adhesion molecules, activation and maturation markers, chemoattractant-and Toll-like receptors. Multiplex technology was exploited to quantify pro-and anti-inflammatory cytokines in plasma and synovial fluids.

Results: The vast majority of synovial fluid neutrophils displayed a strongly activated, hypersegmented phenotype with decreased Lselectin (CD62L) expression and increased numbers of nuclear lobes, upregulation of adhesion molecules CD66b, CD11b and CD15 and downregulation of chemokine receptors CXCR1/2. An elevated percentage of CXCR4-expressing aged neutrophils was detected in synovial fluids from patients. Strikingly, significant percentages of synovial fluid neutrophils showed a profound upregulation of atypical neutrophil markers, including CXCR3, ICAM-1 and HLA-DR. Conclusion: Our data indicate that neutrophils present in inflamed joints of JIA patients are strongly activated cells with elevated proinflammatory and antigen presenting potential. This detailed molecular analysis supports the notion that a complex intertwining between these innate immune cells and adaptive immune events drives JIA.

None declared

The main factors, associated with incomplete vaccination againts measels, parotitis, rubella and diphtheria in 170 juvenile idiopathic arthritis patients N. Lyubimova 1 , I. Objectives: The aim of our study was to evaluate the rate and the main factors of incomplete vaccination against measels,parotitis, rubella (MMR) and diphtheria in JIA patients. Methods: In the present study were included data 170 JIA(55 boys and 115 girls)aged from 2 to 17 years,who received scheduled vaccination before the age of 2 years and before JIA onset against measles,parotitis,diphtheria and rubella.Incomplete vaccination means the reduced number of vaccine to age.In all patients the Ig G anti-vaccine antibodies levels were detected with ELISA.JIA categories were:oligoarthritis -73,polyarthritis -61,systemic-16 and enthesitisrelated arthritis-20.Data presented with median and 25%>75% Results: Incomplete vaccination against MMR was in 50 (42%)diphtheria in 85 (50%) of the JIA patients. The main differences in the studied groups are in the table.There were no differences in sex,JIA categories and treatment, except biologics compare to complete and complete vaccination in all vaccines.No differences in antimeasels(p=0.18),antiparotitis (p=0.1) and anti-rubella(p=0.17)vaccination between complete and incomplete vaccination group.Number of patients with protective level of anti-vaccine antibodies was similar, except parotitis(70% vs 84.2%, p=0.035).The anti-diphtheria antibodies IgG level was lower in the patients with incomplete vaccination group (0.07 IU/ml [95%CI:0.03; 0.22] vs 0.2 [95%CI:0.06; 0.4], p=0.001)as well as number of patients with protective level(34% vs 54%, p=0.002). In the multiple regression model only JIA onset age(p=0.00001)and methotrexate treatment duration(p=0.003) were predictors of incomplete vaccination against MMR and methotrexate treatment duration(p=0.005) and biologic treatment(p=0.05) for diphtheria incomplete vaccination.Incomplete MMR vaccination influence on the maintenance of the protective anti-parotitis level(p=0.036)in regression model.In correlation analysis the number of vaccination influences on anti-diphtheria antibodies level(p=0.017)and number of patients with protective level of anti-diphtheria antibodies(p=0.017). The main predictors in logistic regression for incomplete vaccination for MMR were:onset age<6 years(OR=7. 8 Conclusion: Younger onset of JIA age, longer duration of JIA and methotrexate treatment, biologics and more than 1 biologics are the main predictors of incomplete vaccination againt MMR and diphtheria.

Introduction: The prevalence of autoimmune thyroid disorders (AITD) has been reported to be higher in patients with juvenile idiopathic arthritis (JIA) in comparison to the general population. Nevertheless, there is a lack of studies investigating risk factors for AITD development in children with JIA.

Objectives: To investigate the co-occurrence of JIA and autoimmune thyroiditis in southern Italy and to identify potential predisposing factors to anti-thyroid antibodies (ATA) positivity in a JIA population.

Methods: A single-centre retrospective study was conducted. All JIA patients admitted to the Pediatric Rheumatology Unit of the University of Naples Federico II, from January 2001 to December 2019, tested for ATA at least once and with a minimum of 6-months follow-up, were included. For each patient, demographic, clinical and laboratory data were extracted from clinical charts. Differences between patients affected by JIA with or without ATA were analyzed. Results: Three hundred thirty JIA patients (247 females; median age 12.5 years, IQR 9.1-16.1) were included in study. Median age at JIA onset was 4 years (IQR: 2.2-7.8). Twenty-three patients [7% (95% CI 4.5-10.

3)] presented ATA positivity. Twenty-one of them (91.3%) were females. Anti-thyroperoxidase was positive in 18/23 patients (78.2%) while 12 patients presented anti-thyroglobulin positivity (52.1%). Both antibodies were present in 8/23 (34.8%). 19 patients showed the typical ultrasound findings of autoimmune thyroiditis, resulting in a prevalence of Hashimoto's thyroiditis of 5.7% (95% CI 3.5-8.8) in our cohort. Three female patients developed subclinical hypothyroidism, whereas one male patient presented subclinical hyperthyroidism. The remaining 19 patients were euthyroid. No statistically significant difference was observed in regard to age of JIA onset, follow-up duration and JIA subtype between the patients with or without ATA. The proportion of females was marginally significantly higher (p=0.059) in the group with ATA positivity compared to children without thyroid antibodies (91.3% vs 73.6%, respectively). 56.5% of patients with ATA showed ANA positivity compared to 37.5% of patients without ATA (p=0.07). Family history for AITD was significantly higher in children with thyroid antibodies positivity (p= 0.01). Anti-TNF-alpha inhibitors were administered in only 3 children (13%) with thyroid antibodies before their detection compared to 35 .5% of patients without thyroid antibodies (p=0.028). Multivariate regression analysis showed that patients with a family history for AITD were about four times more likely to develop ATA (OR 3.75, 95% CI 1.401-10.017, p=0.008) and confirmed that ATA positivity is less likely to occur in patients undergone anti-TNF-alpha therapy (OR 0.127, 95% CI 0.031-0.518, p=0.004).

Conclusion: A high prevalence of ATA positivity and Hashimoto's thyroiditis in patients with JIA was found in our wide southern Italian cohort. As expected, a positive family history of AITD was found to be associated with a higher risk to ATA development during the follow-up. This finding supports the usefulness of an active screening for AITD in JIA children, in particular in patients with relatives affected by thyroid disorders. Notably, patients treated with TNF-alpha inhibitors resulted less likely to develop thyroid antibodies. Further studies are needed to investigate the effect of anti-TNF-alpha therapy on thyroid autoimmunity in JIA.

Introduction: The knee is considered by far the joint most frequently affected at JIA onset. Nonetheless, JIA onset may present with unusual musculoskeletal involvement, eventually leading to a delay in the diagnosis and treatment.

Objectives: To identify the type and number of musculoskeletal sites affected at JIA onset in consecutive patients seen at the study center in an 8 years period.

Methods: Records of patients with new diagnosis of JIA from June 2012 to May 2020 available information in the medical history and standardized joint assessment at diagnosis, were retrospectively reviewed. Systemic JIA subtype according to ILAR classification criteria were excluded. Demographic and clinical features, including the type and number of joints at disease onset and diagnosis, were registered. Data were analyzed through descriptive statistics. Results: Of a total of 333 Caucasian patients included in the study (75.7% females), 241 patients (72.4%) had oligoarthritis, 79 (23.7%) RF-negative polyarthritis, 7 (2.1%) RF-positive polyarthritis, 1 (0.3%) psoriatic arthritis, 5 (1.5%) enthesitis-related arthritis (ERA). Antinuclear antibody (ANA) were positive in 188 patients (56.5%). The median age at onset was 4.8 years (IQR 2.3-9.3). At diagnosis 103 (30.9%) patients had only 1 active joint, 143 (43.0%) had 2-4 active joints, 87 (26.1%) had ≥ 5. As expected the knee, the tibiotalar and the wrist were the most frequently affected joints (77.2%, 41.1%, 21.0%, respectively); cervical spine was involved only in patients with polyarthritis (n=13). Notably, of 103 patients with monoarthritis at diagnosis 98 presented with large joints involvement, among which n=2 isolated elbow and n=2 isolated wrist, and 5 with small joints involvement (Table 1) . No sufficient data were available regarding the involvement of tendons and bursae, since the standard joint assessment form did not include them. Nonetheless, additional 4 patients, not included in the sample analysis, had isolated tenosynovitis involvement at diagnosis (n=1 both-sided ulnar extensor tendons; n=2 isolated tenosynovitis of the flexor digiti proprius; n=1 tenosynovitis of 2 flexors digiti proprii). Conclusion: Our study confirms the knee, the tibiotalar and the wrist as the most frequently affected joints at JIA diagnosis. On the other hand, musculoskeletal sites, such as small joints of hands and feet, the hip and the shoulder, usually involved in polyarticular JIA, can be the site of disease presentation in oligo-and also mono-articular JIA. Further, JIA may present with isolated tendon involvement. Our results foster not to delay JIA diagnosis in persistent synovitis occurring in infrequent joints and to include musculoskeletal sites, other than joints, in the standard articular evaluation. This could be realized by merging clinical and imaging (i.e. ultrasound) musculoskeletal examinations in the same assessment.

None declared Introduction: Treatment response in JIA is currently viewed as a binary outcome: response or non-response. However, JIA is a heterogeneous disease and it is likely that different, identifiable subgroups of children and young people (CYP) may demonstrate different patterns of disease following treatment. Identifying these response subgroups can assist the tailoring of stratified treatment approaches in JIA.

Objectives: To identify subgroups of CYP defined by different trajectories of juvenile arthritis disease activity score (JADAS) components following methotrexate (MTX) initiation for JIA. Methods: MTX-naïve CYP with JIA were selected if enrolled prior to January 2018 in the BSPAR Etanercept Cohort Register or the Biologics for Children with Rheumatic Diseases Study at point of starting MTX. JADAS components (active joint count, physician's global assessment (PGA, 0-10cm), parental global evaluation (PGE, 0-10cm) and standardised ESR (0-10) were calculated based on data collected in the year following MTX initiation. Multivariate group-based trajectory models were used to explore MTX response clusters across the different JADAS components, which were log1p transformed for analysis. Optimal models were selected based on a combination of model fit (BIC, relative entropy, average posterior probabilities), parsimony and clinical plausibility. Clinical and demographic characteristics and achievement of ACR Pedi 30/90 by six months were compared across identified groups. Results: Of 658 CYP selected, the majority were female (68%) and of white ethnicity (86%), with RF-negative JIA the most common disease category (35%). Six subgroups of CYP were identified with differing patterns of disease activity following MTX initiation. Two groups improved across all JADAS components: Fast improvers (11%), and Slow improvers (16%). Persistent PGA (8%), and Persistent PGE (13%) groups maintained one persistent disease feature but otherwise improved. One group relapsed (7%) and a final group had persistent disease overall (44%). There were no differences in active joint counts at MTX initiation between subgroups and all ILAR categories were represented across each subgroup. However, CYP in persistent disease and slow improver groups had higher CHAQ, PGA and PGE scores at MTX initiation. Those with persistent disease were also older at MTX initiation. The majority of CYP fulfilled ACR Pedi 30 response (>60% across every group). ACR Pedi 90 achievement was low at 6 months for slow improvers (30%) and high in the relapse group (68%). Between 41% and 73% achieved ACR Pedi 90 response in groups with persistent disease in one JADAS component.

We identify different patterns of disease activity within CYP initiating MTX, suggesting a simple responder/non-responder analysis at a set point may be over-simplistic. Commonly used response measures did not adequately describe these heterogeneous response patterns. Understanding both clinical factors associated with, and biological mechanisms underpinning, these subgroups would aid stratified medicine in JIA.

Introduction: Despite modern treatment and improved disease control, pain is the most common complaint in juvenile idiopathic arthritis (JIA). Knowledge about pain thresholds and pain sensitivity among young adults with JIA is sparse. Objectives: To study pressure pain thresholds (PPTs) in young adults with JIA, 16 years after disease onset compared with controls. Methods: Consecutive newly diagnosed children with JIA and a disease onset between 1997-2004 from Central Norway, were included in this prospective population-based long-term follow-up study. Children with onset 1997-2000 were part of the Nordic JIA cohort 1,2 . Age-and sex-matched controls were drawn from the National Population Register of Norway. Inactive disease and remission were defined according to Wallace 3, 4 . At the follow-up between 2015-17, data from a clinical examination and blood tests were included in addition to an investigator-blinded quantification of PPTs. A digital algometer was used to manually apply pressure three times with an even rate at the upper and lower limb. PPTs from JIA and controls, and from subgroups of JIA defined by disease status, were compared with multilevel models in STATA.

Results: Among the 96 participants with JIA, 71% were female, median age was 22.7 (IQR 18.7-26.2) years, median disease duration was 16.1 (IQR 14.2-17.1) years, 47% had an oligoarticular disease (persistent or extended), and 45% were in remission off medication.

In the control group, 71% were female and median age was 23.5 (IQR 20.2-26.7) years. Results from the multilevel regression model showed significantly lower PPTs among participants with JIA compared to controls (Table 1 ). In the JIA group, participants with inactive disease had lower PPTs than both JIA in remission off medication and JIA with active disease ( Table 1 ). The results were consistent for both upper and lower limb. Conclusion: In this long-term follow-up study of young adults with JIA, we found significantly lower PPTs compared to controls. This may indicate that young adults with JIA have altered pain sensitivity possibly due to accumulated earlier pain experiences. Introduction: Juvenile Idiopathic Arthritis (JIA) represents the most common chronic rheumatic disease in childhood. Non-steroidal antiinflammatory drugs (NSAIDs) and intra-articular steroids are the first line treatment for JIA. Systemic steroids, disease modifying antirheumatic drugs (DMARDs) and biologic drugs are used in children with severe disease. It is not possible at onset of disease to predict when a child can suspend pharmacological treatment, so children affected from JIA have to continue pharmacological treatment for several months or years. Anecdotal reports showed that rarely JIA could present renal involvement due to uncontrolled inflammation or to long exposure to drugs. Objectives: Because no cohort studies investigating renal injury in children with JIA are available, we designed this kind of study in our population. Methods: We retrospectively evaluated 110 patients suffering from JIA. JIA diagnosis was made according to ILAR criteria, treatment was assigned with ACR recommendations. For each patient we recorded the type and the duration of pharmacological treatment and the presence of renal injury. Renal injury was defined by the presence of hypertension (systolic and/or diastolic blood pressure >95 th percentile for age, sex and height), proteinuria (persistentconfirmation within 3 months-urinary protein/creatinine ratio>0.5 mg/mg for children <2 years old and >0. 2 Introduction: Juvenile idiopathic arthritis (JIA) is a pediatric rheumatic disease with partially unknown etiology and pathogenesis. Neutrophils are the most common immune cell present in synovial fluid from inflamed joints in oligoarticular JIA, but the role of neutrophils in the immunopathogenesis of oligoarticular JIA has not been investigated.

Objectives: To characterize neutrophil phenotypes and effector functions in the circulation and in the inflamed joint during active arthritis in children with oligoarticular JIA. Methods: Paired samples of blood and synovial fluid from 17 children with oligoarticular JIA were investigated regarding surface markers, phagocytic ability and oxidative burst. Healthy blood neutrophils exposed to cell-free JIA synovial fluid and healthy oral cavity neutrophils were studied as controls for synovial fluid exposure and transmigration respectively. Results: Synovial neutrophils had a shifted phenotype, characterized by high surface levels of neutrophil activation markers CD11b and CD66b, and mannose receptor CD206 and decreased levels of adhesion molecule CD62L compared to circulating neutrophils. In comparison to oral cavity neutrophils, synovial neutrophils had higher levels of CD11b and a different overall phenotype, suggesting that the phenotype shift in synovial compared to circulating neutrophils is not dependent on transmigration alone. JIA synovial fluid in itself induced activation of healthy blood neutrophils, measured as increased CD11b levels. Synovial fluid neutrophils had impaired ability to phagocytose opsonized E. coli and to produce oxygen radicals upon neutrophil activation with phorbol-myristateacetate (PMA) compared to circulating neutrophils. The impaired effector functions in synovial neutrophils was not dependent on the synovial fluid alone, as addition of cell-free synovial fluid to blood neutrophils did not alter phagocytosis and oxidative burst. Conclusion: JIA synovial fluid neutrophils are activated, have a "polarized" phenotype and impaired effector functions compared to neutrophils in the circulation. This study will help bridge the current knowledge-gap regarding the role of neutrophils in the immunopathogenesis in oligoarticular JIA. Methods: A case report is described. Data was extracted from the medical chart of the patient and a literature review was undertaken.

Results: A 7-year-old girl was transferred to our tertiary center after being admitted for prolonged intermittent fevers, abdominal pain, fatigue and polyarthralgias. On examination, there was symmetrical proximal muscle weakness, a vasculitic lower limb rash, facial erythema with eyelid edema (Fig. 1 ) and oral mucositis. Initial laboratory exams revealed pancytopenia, high muscle enzymes, increased erythrocyte sedimentation rate with moderately elevated reactive C-protein, and hypocomplementemia. She also had non-nephrotic proteinuria, without hematuria.Further investigations showed a positive direct antiglobulin test, antinuclear antibodies, antidouble-stranded DNA, anti-Mi 2 and anti-Ku. Serositis (pericardial and pleural effusions, ascitis) and hepatosplenomegaly were present. Lower limb MRI documented diffuse muscle edema. The diagnosis of an overlap syndrome of jSLE and IIM was established. While being treated for concomitant bacteremia, the patient became ill-appearing, with persistent fevers, worsened cytopenias, low fibrinogen and high ferritin and triglycerides, and a Macrophage Activation Syndrome (MAS) diagnosis was assumed. The patient received antibiotics and intravenous immunoglobulin, followed by methylprednisolone pulses, IV cyclosporine (CYC), hydroxychloroquine and supportive therapy with progressive improvement. Due to hypertension (possibly related to CYC) and persistent proteinuria a renal biopsy was performed showing class IV lupus nephritis. After achieving clinical stability, CYC was switched to mycophenolate mofetil as an induction treatment, which is ongoing. Conclusion: IMM with SLE OS is uncommon, and has seldom been described in children. In addition to fulfilling SLE criteria, our patient had clinical, laboratory and imagiologic evidence of IMM. The presence of myositis specific antibodies (especially anti-Mi 2) further supports the diagnosis of an OS rather than an atypical presentation of a lupus myopathy. Juvenile dermatomyositis appears to be the IMM subtype -it is associated with anti-Mi 2, and mild heliotrope and eyelid edema are compatible. Facial rash sparing the nasolabial folds is more suggestive of SLE. MAS is a rare but life-threatening condition that should be suspected in rheumatologic conditions and might be triggered by infections or disease flares. Its identification may be particularly challenging at presentation, especially in SLE where cytopenias are common. The reported prevalence in adult SLE ranges from 0.9% to 4.6%; disease-specific criteria have been proposed. MAS has occasionally been described in IIM. In a patient with a predisposing condition, persistent fevers and illappearance must always prompt a MAS workup, since early diagnosis and treatment are paramount. Due to an early referral to a pediatric rheumatology center, the patient received a prompt diagnosis and treatment, which probably improved her prognosis. Results: Four of the five patients were female (80%) and all aged between 6 and 10 years. Four of them had calcinosis at the time of diagnosis, although they may have had symptoms for 12 to 18 months prior to diagnosis. Skin involvement was severe requiring multiple systemic and topical therapeutic agents in four out of the five patients -significantly more affected than the muscles. One patient had amyopathic subtype with normal Childhood Myositis Assessment Score (CMAS) throughout. None of them had cardiac involvement. All had weakly positive Anti-Nuclear Antibodies (ANA); but were negative for myositis antibodies except the patient with most severe skin involvement and calcinosis (patient 2), who was positive for Anti-TIF1gamma antibodies. Two of the three patients with calcinosis at onset had Cyclophosphamide as the second line agent (chosen due to calcinosis) following systemic corticosteroids with complete resolution of the lesions after six cycles at 500mg/m2. One patient responded to Infliximab, which failed to work after 20 months, following which Cyclophosphamide was tried with good response. The other two patients were given Cyclophosphamide after they failed to respond to Rituximab, which did work for muscle disease. One patient had recurrent episodes of calcinosis needing surgical curettage despite initial response to Cyclophosphamide and later IVIG. Introduction: Systemic Juvenile Idiopathic Arthritis (sJIA) is a unique form of childhood arthritis. According to current understanding sJIA is primarily driven by innate immune mechanisms at disease onset, but can progress towards chronic destructive arthritis, which can involve T cellular immunity. For yet incompletely understood reasons, sJIA can be complicated by Macrophage Activation Syndrome (MAS), a severe hyperinflammatory condition characterized by a catastrophic cytokine storm resulting in multiple organ failure and high mortality. Objectives: The sJIA/MAS Working Party (WP) aims to promote knowledge and international multidisciplinary collaboration among experts in the field of MAS and sJIA and to foster translational research in order to improve the care and outcome of these patients Methods: Currently 60 PReS members participate to the MAS/sJIA WP. The WP arranges an annual meeting during the PReS Congress, open to all members activities. The MAS/sJIA WP core team frequently report about ongoing activities by email.

Results: Several studies are currently ongoing. A project aimed to establish and validate a risk score for MAS in sJIA patients using routine laboratory parameters of disease activity and severity has already completed the construction phase. Recently, building of a validation cohort comprising data form 182 patients from 10 paediatric rheumatologic centers has been accomplished and is awaiting analysis (Claudia Bracaglia). A second project focused on MAS patients with systemic thrombotic microangiopathy (TMA) has just completed the collection of 27 patients with MAS and TMA from 18 centers in 9 countries and results will soon be published (Francesca Minoia). Furthermore, the MAS/sJIA WP participated in the data collection phase of a project on the development of new criteria for primary HLH (Jan-Inge Henter and AnnaCarin Horne Introduction: Hemophagocytic lymphohistiocytosis (HLH) is an immunological disorder characterized by clinical signs and symptoms of severe uncontrolled inflammation, due to massive release of inflammatory cytokines. A delay in diagnosis is common, and is one of the factors that determine the poor outcome. HLH is classified into primary (pHLH) and secondary (sHLH). It is important to differentiate between the two as management differs. Objectives: To describe the clinical and laboratory profile of HLH in infancy.

Methods: The electronic case files of children (age<1 year) diagnosed with HLH at the AIMS, Kochi, Kerala, between January 2012 and December 2019, was retrospectively reviewed and described. Results: Eight infants, with age range 1.5 months to 7 months, were clinically diagnosed with HLH. All were immunised and had normal development for age. None had a family history suggestive of HLH. Third degree consanguinity was present in parents of patient no.5 and second degree for patient no.7. Duration of symptoms before presentation ranged from 2 days to 68 days. Duration of follow up with us ranged from 12 days to 192 days, for those who expired. All, eight of them, had fever, anemia, thrombocytopenia, hyperferritinemia, transaminitis, raised LDH and CRP. Lymphadenopathy was present only in patient no.4. Before starting specific treatment patient no. 7 had Pseudomonas sepsis, patient no.5 had Roseomonas gilardii infection; patient no.3 and 4 were IgM CMV positive but their PCR was negative. Both of them had received prior blood transfusion. Before making a definitive diagnosis of HLH patients were treated for PUO, sepsis ? cause and acute liver failure. There was a delay in diagnosis for all patients except patient no.7. All of them were treated with HLH 2004 protocol with modification according to clinical status of the patient. Later, broad spectrum antibiotics, antifungals and antivirals were used for all. Anakinra was tried for patient no.5. Five patients (pHLH) succumbed to sepsis and MODS and three (one pHLH and two sHLH) are continuing follow up. HSCT was not done in any of them. Other clinical features are shown in Table 1 . Conclusion: Making a timely diagnosis of HLH is difficult. Differentiating pHLH from sHLH is very important as the management differs. Genetic testing should be done for all infants with HLH. Negative genetic study doesn't rule out pHLH. The only curative treatment for pHLH is HSCT. sHLH infants, once their primary condition is treated, can have normal survival. Hyperbilirubinemia, splenomegaly, neutropenia, hepatomegaly, tissue hemophagocytes and hypertriglyceridemia were more common in pHLH. Health, Kolkata diagnosed as having MAS, admitted between July 2008 and April 2020 was tabulated and retrospectively analyzed . Objectives: To evaluate the clinical features, laboratory findings and outcomes in pediatric MAS, assess the response to different pharmacological therapies, and finally to identify possible factors associated with an unfavourable outcome. Methods: The data of patients diagnosed with MAS over the study period was analyzed for the clinical and laboratory features, treatment details, response to therapy and outcome. Results: 35 patients were diagnosed as having MAS. Primary illness was sJIA in 29 (82%), SLE in 5 (14%) and Kawasaki Disease (KD) in 1(4%). All had fever with varying degrees of multi systemic involvement. Hyperferritinemia was universally present. In the absence of Anakinra in India, pulse methylprednisolone with Cyclosporine was used for treating the majority.10 patients (28.5%) expired. Patients on biologics and steroids can present with a silent MAS which may be difficult to diagnose. Conclusion: MAS is a near fatal complication with protean manifestations and multi organ dysfunction. Hyperferritinemia is characteristic, higher values being associated with increased mortality. Patients resistant to steroids and cyclosporine had a poor prognosis. Early recognition with aggresive management forms the backbone of a successful outcome as reflected by improved prognosis over successive years. Late presentations with multiorgan dysfunction are associated with the poorest outcomes. Methods: Case report's description Results: A two-year-old boy presented with one month history of fever associated with limping gait, cervical lymphadenopathy and skin rash. Laboratory tests showed elevation of inflammatory markers and ferritin. By exclusion criteria, sJIA was diagnosed and steroid therapy started. After a soft tissue bacterial infection, fever relapsed and laboratory tests were consistent with MAS (day 1): Hb 8.5 g/dL, PLT 44000/mm3; FDP 1522 ug/L, CRP 100 mg/L, ferritin 2200 ug/L. High doses intravenous metilprednisolone and oral Cyclosporin A (CSA) were started. On day 2 he presented a Systemic Capillary Leak Syndrome and acute myocarditis. He was admitted into the pediatric intensive care unit (PICU) where intravenous immunoglobulin and subcutaneous Anakinra (ANA) were added. On day 4, due to an Introduction: Sjögren's syndrome is a systemic autoimmune disease characterized by dry syndrome and lymphocytic infiltration of the exocrine and extraglandular glands. Pulmonary involvement in primary Sjögren's syndrome occurs in 9-20% of patients, with very heterogeneous manifestations, and occasionally as an initial mani-festation¹. Diffuse interstitial lung involvement is one of the most characteristic pulmonary manifestations and the most frequent subtypes in lung biopsy are interstitial lymphocytic pneumonia and nonspecific interstitial pneumonia².

Objectives: 14-year-old girl presented to our hospital because of bilateral interstitial involvement with ground glass areas in lower lobes of both lungs on thorax and abdominal CT scan after for kidney stones follow-up. The patient had grade 1 mMRC dyspnoea and dry cough but denied having symptoms of arthralgia or arthritis, photosensitivity, oral and genital ulcers, Raynaud's phenomenon or episodes of dry mucosa. She had no history of autoimmune disease nor family antecedents of any autoimmune disease. A physical examination disclosed no finger clubbing or swollen superficial lymph nodes but indicated crackles on pulmonary auscultation. Laboratory work showed elevated acute phase reactants, positive rheumatoid factor, positive antinuclear antibodies (1/ 40), positive cytoplasmic antineutrophil antibodies (1/320) and IgG and IgA hypergammaglobulinemia. An examination for autoantibodies were negative for anti-SS-A, anti-SS-B, anti Jo-1, anticentromere and anti-scl-70 antibodies. Iontophoresis with pilocarpine and 6-minute walk test was also normal. Pulmonary function tests demonstrated a mild restrictive impairment and a reduced percent diffusion capacity for carbon monoxide of 55%. Fibreoptic bronchoscopy showed acute inflammation in bronchial mucosa. Flow cytometry of bronchoalveolar lavage and cytology showed lymphocytosis with a 15% of CD4 and 85% of CD8 lymphocytes in bronchoalveolar lavage fluid. Finally, a transbronchial lung biopsy lead to a definitive diagnosis, showing mixed interstitial inflammation and lymphocytic follicular hyperplasia with formation of germinal centers, suggestive of a lymphoid interstitial pneumonia of unreleased autoimmune etiology. Throughout time, the patient reported progression of her symptoms with increasing dyspnoea, persistent dry cough, xerostomia and arthralgia. Schirmer and Rose Bengal dye test were negative, and a salivary gland biopsy showed interstitial plasmacytosis and no IgG4 plasma cells expression which suggested Sjogren's disease. A high resolution computerized axial tomography was requested, suggesting organizing pneumonia in the context of Sjogren's disease.

Methods: Several studies indicate that lung involvement in Sjögren is more frequent in advanced stages of the disease and rarely as an initial manifestation. Sjögren's syndrome in paediatric age is rare and the subtype of secondary Sjogren's is the most common. The course is longer, and the symptoms are more heterogeneous than in adulthood 5 . The diagnosis in children is delayed, because children less frequently report dryness and frequently present with extraglandular clinical features suggestive of other autoimmune diseases. A systematic review on primary Sjögren's syndrome in male and paediatric population reported a 2.4% of pulmonary involvement in paediatric patients. 6 Pulmonary involvement is associated with an increase in the mortality of patients with Sjögren's, therefore, it is essential to periodically monitor patients with respiratory symptoms, making an early diagnosis and treatment of the disease. Results: -Conclusion: We present a case of a patient with childhood Sjögren's disease with atypical onset of disease with lung involvement. Introduction: Sarcoidosis is a multi-system disorder. Little is known about its pathogenesis. In children, the early onset sarcoidosis phenotype including Blau syndrome is more often seen. 1, 2 The diagnosis of sarcoidosis is confirmed by demonstrating a typical non-caseating granuloma on a biopsy specimen. Other granulomatous diseases should be excluded, in particular mycobacterial infections, Crohn's disease and immunodeficiencies. The clinical presentation may vary depending on the organs involved and the age of the patient. 3, 4 Objectives: We are reporting the case of a boy with a presentation of bone sarcoidosis at a young age. This is a rare phenotype in children.

Methods: Clinical details were retrospectively collated using routine clinical records. Confirmation of diagnosis was confirmed with bone biopsy.

Results: A 5 year old non-identical twin boy of Ghanaian descent born in the UK had a slowly growing, painless frontal bone mass which started to develop from 7 months of age. He was developmentally normal, with no history of fever, rashes or joint pains. Examination findings revealed frontal bossing while the remainder of the musculoskeletal examination was normal. There was no evidence of rashes, hepatosplenomegaly and ocular examination was normal.

The patient was initially referred for neurosurgical review with suspected fibrous dysplasia, after an initial MRI scan of the head revealed abnormal marrow signal and expansion of the frontal bone, with no soft tissue swelling. However, the CT scan of the calvarium was not suggestive of fibrous dysplasia. Consequently, bone biopsy was performed demonstrating inflammation with granuloma formation. He was referred to Infectious Diseases and Rheumatology. There was no travel history and no TB contact. QuantiFERON TB was negative. Infectious work-up was negative especially for mycobacterial infections. Rheumatology work-up identified on skeletal survey another bone location: a well-defined lytic lesion in the right distal fibula that was biopsied. Infection cultures and PCR were negative. Histopathology identified fibrous tissue and poorly formed granulomas. Laboratory investigations revealed a mild microcytic anaemia with iron deficiency and eosinophilia. He had normal serum calcium and vitamin D and his ESR was 25 mm/hr. ANA, ANCA and rheumatoid factor were negative, and complement C3 and C4 were normal. His serum Angiotensin Converting Enzyme (ACE) level was raised at 125 nmol/ ml/min (normal <40 nmol/ml/min). Investigations revealed mild renal impairment with normal urinary tests including normal calcium, protein and tubular proteins. Ultrasound of the kidneys was normal. Chest X-ray was normal. Lung function was performed and was normal. DLCO couldn't be performed due to low lung volume. Vascular and inflammation genetic panel identified a variant in the NEMO gene. Functional studies excluded NEMO deficiency and patient did not display any of the clinical features. However, a pattern of dysregulated T cells response was identified. He was treated with oral steroids and methotrexate. The oral steroids were successfully weaned off. He has been successfully treated with Methotrexate 10mg s/c to initially stabilise disease with no bone growth, and had no significant side effects. Repeat MRI 2 years later showed increased burden of disease with other newly affected sites however, including the right femoral diaphysis and signal changes in the left tibial metaphysis. Based on the MRI and increasing musculoskeletal pain, decision was made to escalate to anti-TNF (adalimumab) with good clinical response. Conclusion: Bone sarcoidoisis is rare in children but this should be considered in the differential diagnoses when granulomatous inflammation is identified on histopathology. Response to steroids and methotrexate is usually good but some patients will need escalation to anti-TNF.

The most worrisome non-rheumatic condition causing persistent night pain in children which closely mimics arthritis is malignancy 1, 4 . It is vital to pick up subtle clues at an early stage especially in absence of hematological manifestations , organomegaly and lymphadenopathy.

To reveal early clinical clues in pediatric patients with predominant musculoskeletal (MSK) night pains who were initially diagnosed as suffering from some form of chronic arthritis but ultimately turned out to be affected by malignancy. Methods I gathered a data of five pediatric patients fulfilling above mentioned criteria who were seen at Dev Children's Hospital between January 2019 and March 2020. It included demographics, clinical presentation and laboratory results.

All above cases reemphasize the need for an extremely detailed history pertaining to characteristics of pain & pattern recognition in pediatric rheumatology. Prolonged fever , persistent MSK night pain, persistent limp, upper limb and hip joint involvement which is unlikely for JIA at onset are proven to be the earliest subtle clues which should not be missed. 1 Other constitutional symptoms, respiratory, cardiovascular, ophthalmologic or osteoarticular involvement were absent. Growth was unaffected. Auditory tests were normal. Systemic antibiotic treatment and local steroids were ineffective. Laboratory findings were unremarkable, with only mild elevation of ESR (28mm/1 st hr). ANA and ANCA were absent in repeat meausrements (3 months intervals). Cardiovascular disease was excluded. Abdominal US was normal. On the basis of relapsing bilateral auricular chondritis and confirmatory histological findings revealing inflamed cartilage from the pinna of the ear with chondrocyte degeneration, perichondrial infiltrates of lymphocytes, plasma and polymorphonuclear cells and replacement of cartilage with fibrous tissue perivascular infiltrates of polymorphonuclear cells and lymphocytes, relapsing polychondritis was diagnosed. One month NSAIDS trial, pending histology results was ineffective. Methotrexate SC and steroids 1mg/kg/d gradually tapered over a 3-month period were given with significant improvement of auricular inflammation and normalization of markers of inflammation. Auricular chondritis worsened after steroid withdrawal and adalimumab was added to treatment with significant improvement of auricular inflammation in 2 months. In the following 8 months auricular chondritis relapsed during URIs with mild elevation of ESR (25mm 1st hr) and CRP (13 mg/l). After 15 months of treatment, in an effort to prolong the intervals of adalimumab administration, bilateral auricular chondritis relapsed. After 24 months of MTX and 21 months of adalimumab administration inflammation was put in complete remission. The following year no flares or involvement of other systems were observed, under methotrexate and adalimumab treatment.

Conclusion: In this patient isolated auricular relapsing polychondritis was unresponsive to NSAIDs. Steroids and methotrexate greatly improved inflammation but did not induce complete remission. Complete remission was achieved by addition of adalimumab to methotrexate treatment, which also allowed for steroids discontinuation.

None declared

First ever single center study revealing spectrum of rheumatic diseases in 114 children from an Indian State of Gujarat D. B. Pandya, on behalf of Dr Mehul Mitra, Pankaj Buch, Sonal Shah,

There is very limited information and awareness about pediatric rheumatic and immunodeficiency diseases amongst primary physicians 1, 2, 3 in Gujarat and to make this matter even worse, we are not having a single exclusive pediatric rheumatology and immunology centre for a population of around 60 million.

To guesstimate a status of children with rheumatic and immunodeficiency diseases in Gujarat and spectrum of these diseases at Dev Children's Hospital. Methods I gathered a retrospective data of 174 patients who attended Dev Children's Hospital between January 2019 and January 2020. Out of these, 114 children with confirmed diagnosis of inflammatory rheumatic diseases and suspected primary immunodeficiencies were included. Patients with non-inflammatory musculoskeletal(MSK) pains and non-rheumatic diseases causing MSK pains were excluded. My collected data included referral details, demographics, clinical presentation, laboratory results and diagnosis.

Majority of the cases were referred by pediatricians, orthopedicians, hemato-oncologist and general physicians. Main reasons for referral were joint involvement , undiagnosed fever , multisystem disease and elevated inflammatory markers. Many physicians had put a diagnosis like rheumatoid/rheumatic arthritis, autoimmune disease or connective tissue disease. Almost 80% of patients had been evaluated with RF, ASO titer, ANA and joint imaging irrespective of clinical pattern by their primary physicians before referral. Fever , MSK involvement, extreme fatigue, constitutional symptoms, skin and mucosal involvement were prominent complaints noted by me. Family history of rheumatic, primary immunodeficiency (PID) or consanguinity was found in 1/3 of patients. Anemia of chronic disease, elevated ESR and thrombocytosis were almost universal laboratory findings in our cohort.

Rheumatic diseases in children are not anymore rare but due to lack of expertise and awareness , these children are not getting diagnosed. Many cases were advised unnecessary rheumatological investigations even before referral. Results: A 10-year-old female patient was referred to the rheumatology clinic at our hospital with a previous history of fever of 39°C (102.2ºC), loss of appetite, and acute polyarthritis of wrist, knees, and ankles. At that time, laboratory exams revealed a hemoglobin of 11.1 g/dL, C reactive protein 78.6 mg/L, and antistreptolysin O titers of 400 UI/mL (normal range <200UI/ml. Clinical symptoms were relieved only after using NSAIDs. After 6 months, the patient returned to our hospital with a 7-month history of weight loss and claudication related to pain and daily morning stiffness (15 minutes) on her right ankle. New laboratory findings demonstrated positive antinuclear antibodies 1:320, negative rheumatoid factor, and alpha-1-acid glycoprotein of 171 mg/dL (normal range: 44-113mg/dL). Clinical signs suggestive of chronic arthritis with exuberant swelling of the ankles were observed on physical examination (figure A). She was screened for tuberculosis (TB) and had a positive (18mm) tuberculin skin test (figure B). Chest CT revealed infiltrative soft tissue mass in the posterior mediastinum, with homogeneous contrast enhancement (figure C). Magnetic resonance imaging of both ankles was performed and demonstrated bilateral and symmetrical tibiotalar arthritis and prominent tenosynovitis of extensors, flexors, and fibularis tendons (figure D). Right ankle synovial biopsy revealed no granulomas and joint fluid culture was negative for Mycobacterium tuberculosis, confirming reactive arthritis (Poncet's) and tenosynovitis, that may follow mycobacterial infection with no infective agent in the joints. Conclusion: To our knowledge, there is no report of Poncet's disease associated with inflammatory tenosynovitis, showing the particularity of this case. The patient's symptoms resolved after two months of anti-TB therapy.

Introduction: CACP is characterized by congenital or early-onset camptodactyly (usually bilateral); non-inflammatory arthropathy (more frequently in the wrists, knees, ankles, elbows, and hips); coxa vara (reduction of the angle between the neck and shaft of the femur); and non-inflammatory pericardial effusion (a late manifestation, less frequently reported). Recognizing the radiological aspects of this syndrome and differentiating it from JIA is crucial since CACP has no effective treatment and JIA is usually treated with NSAIDs and methotrexate (2, 3) .

Objectives: To report a rare case of CACP syndrome mimicking JIA. Methods: Case report and literature review.

Results: A 5-year-old male patient presented with arthropathy characterized by painless progressive swelling and restricted movement of the hands, hips, knees, and ankles since the first year of life. He had a family history of camptodactyly from his paternal grandfather. On physical examination, symmetric camptodactyly of the hands and feet was observed (A). He had no history of rash or weight loss and inflammatory markers were unremarkable. The echocardiogram was normal. The pelvic radiograph showed a widening of the joint space and bilateral coxa vara. Magnetic resonance imaging (MRI) of the hips (B) and knees (C) was performed and depicted large joint effusions (arrows, B and C) with normal synovial thickness and mild synovial enhancement in all joints, without bone marrow edema-like signal. A synovial biopsy of the knee was performed and revealed mild synovial hyperplasia without inflammatory cells. The patient was diagnosed with camptodactyly-arthropathy-coxa vara-pericarditis syndrome (CACP -OMIM 208250), a recently described genetic disorder with no gender predominance identified to date (1). Conclusion: An important differential diagnosis of CACP is juvenile idiopathic arthritis (JIA), a painful inflammatory chronic arthritis that can cause not only joint effusions due to synovial inflammation, but Arthritis was the most frequent extraglandular manifestation. Renal tubular acidosis represented the typical expression of renal involvement (19 cases). Neuromyelitis optica and aseptic meningoencephalitis (6 and 9 cases, respectively) were the most typical neurologic manifestations. Two cases of interstitial lung disease and one of pulmonary hypertension were reported. Almost all patients had autoantibodies, mostly ANA (200/224 patients) and anti-SSA/Ro (170/208 patients). The Schirmer test was performed in less than half of the patients, of whom 62% tested positive. A positive result of minor salivary biopsy was reported in 129/140 cases with available data. Juvenile idiopathic arthritis was the most frequently associated disease, followed by systemic lupus erythematosus (16 and 8 cases, respectively). No significant differences between patients with or without parotitis were found except that patients with parotitis showed increased levels of CRP more frequently than those without it (p= 0.00). Patients with anti-SSA/Ro had more frequently a positive Schirmer test (p= 0.04). The presence of RF was significantly associated with dry mouth (p= 0.00), arthritis (p= 0.00), and rash (p= 0.04). A positive minor salivary biopsy was more common in children with dry eyes than in those without this clinical feature (p= 0.02). Arthritis was more frequent in patients with other diseases than in those with primary SS (p= 0.00). We further investigated SS features according to the age groups (≤ 6 years, 7-11 years, ≥ 12 years). Parotid involvement was inversely proportional to the age and occurred more frequently in younger patients (79% of those ≤ 6 years; p= 0.03). Interestingly, the rate of anti-SSA/Ro positivity increased with age (97% of those ≥ 12 years; p= 0.00). Conclusion: Even though parotitis was the most frequently reported feature, a wide range of clinical manifestations in children with SS has been reported so far. A better knowledge of cSS features will help to pave the way for the development of cSS specific diagnostic criteria.

None declared Introduction: Pachydermodactyly (PDD) is a rare benign fibromatosis, characterized by progressive painless swelling of soft tissue of proximal interphalangeal (PIP) joints without inflammation signs. Generally PDD affects PIP joints of the fingers, rarely of the thumb. The involvement is typically symmetrical, in few cases unilateral. It usually occurs more frequently in young males. Etiology is unknown, but it arises from mechanical stimulation of periarticular skin (i.e repetitive rubbing, interlacing, and cracking of fingers). PDD has to be considered in the differential diagnosis of arthritis (i.e. juvenile idiopathic arthritis, JIA) and many syndromes (i.e. progressive pseudorheumatoid dysplasia). Prognosis is good with cessation of mechanical stimulation 1 The recurrent paroxysmal appearance of inflammatory lumps (local erythematous tender swellings, which partially respond to antiinflammatory agents), accompanied by elevated inflammatory markers during flares, suggest that FOP may be an autoinflammatory disease. The episodic formation of bone, often following a trivial injury, suggests that innate immune-related triggers induce tissue transformation through the BMP pathway. Moreover, interleukin-1β (IL-1β), a well-known mediator of the innate immune system, has been linked to HO and mineralization in mesenchymal stem cell cultures derived from human bone marrow. We hypothesized that treating FOP patients with anti-IL-1 agents could help ameliorate the progression of this devastating disease. We report our experience treating two FOP patients with anakinra and canakinumab.

Objectives: To decrease the frequency of FOP paroxysms, and/or limit the symptoms and extent of residual lesions, by using anti-IL-1 agents.

Methods: Patients' data and blood IL-1 levels were analyzed to characterize the efficacy of anti-IL-1 treatments in ameliorating the natural progression of FOP.

Results: A 13.5 year old boy and a 5 year old girl were diagnosed with FOP, both clinically and genetically (the typical R206H mutation was found). Various treatments, including high-dose corticosteroids, pamidronate infusions, celecoxib, monteleukast and sirolimus, did not change the course of the disease.

Both patients are receiving canakinumab (the male patient was initially treated with anakinra). The male patient has been treated for over 2 years. Flare rate was markedly reduced from one new lump every 8 days to approximately one every 25 days ( Figure 1 ). The lumps involved in almost all of these flares are the same: at the left scapular base and within the sternocleidomastoid muscle. The female patient has been treated for a year, and has not experienced any HO flares during canakinumab treatment. Temporarily withholding canakinumab in both patients, led to serious flares 8 weeks after the last dose. Notably, while undetectable levels of IL-1β (<0.125 pg/ml) were found in the three plasma samples obtained from the male patient during treatment with anakinra or canakinumab, high levels (up to 21.52 pg/ml, about 90-fold higher compared to average levels measured in healthy controls) were found in his plasma samples collected during the flare ( Figure 2 ). In contrast, IL-18 and IL-6 plasma levels, measured before, during and after withholding treatment, were comparable or slightly higher than those observed in healthy controls ( Figure 3A , B). Conclusion: We report here, for the first time, that anti-IL-1 agents were found efficacious in treating two FOP patients. We also found markedly increased IL-1β levels during flares, which normalized following the treatment. We suggest a role for IL-1β in the pathogenesis of this disease. Although it is too soon to conclude whether FOP may be included under the umbrella of auto-inflammatory syndromes, anti-IL-1 agents can be effective in ameliorating the natural progression of FOP. Introduction: Musculoskeletal symptoms are one of the common reasons for applying to rheumatology departments in general practice 1 . Although inflammatory causes are generally considered in the foreground, it is known that non-inflammatory causes including genetic diseases may also be responsible. The absence of signs of inflammation (morning stiffness, redness, tenderness) and normal inflammatory markers in laboratory findings may support nonrheumatologic diseases 2 .

Objectives: To present genetic disorders that can mimic rheumatologic symptoms and to answer when genetic diseases should be considered in the differential diagnosis in patients presenting with rheumatological complaints. Methods: We retrospectively evaluated 60 patients who applied to Hacettepe University pediatric rheumatology department with musculoskeletal compliants between January 2015 and December 2019 and had been consulted to genetics departmant. The rate and degree of consanguinity, clinical diagnosis, indication for consultation, accompanying musculoskeletal and other findings had been recorded. The diagnosis of genetic diseases were based on physical examination, radiological evaluations and genetic analysis.

Results: A total of 60 patients, 19 boys (31.6%), with a mean age 12.46 ± 1.41 years were included in the study. The rate of consanguinity was 25.0%. The most frequent referral to the genetic department was the presence of skeletal anomalies (n:12) such as camptodactyly, clinodactyly, and bone shortness accompanying joint findings. Other causes include short stature (n:4), joint deformity (n:5), joint hyperlaxicity (n:10), dysmorphic findings such as atypic facial appearance (n:9), accompanying diseases that may be part of a syndrome (n:11), genetic diagnosis suspicion according to the results of radiological examination (n:4) and joint findings without clinical and laboratory signs of inflammation (n:5). Distribution of joint involvement in 20 patients with genetic disease were hands, knees, and hips respectively. In the laboratory evaluation of patients presenting with joint swelling and arthralgia, acute phase reactants (erythrocyte sedimentation rate and C-reactive protein concentrations) were within normal reference values. One third of the patients (33.3%) had a final diagnosis of a genetic disease. The diagnoses of these patients were as follows; CACP (camptodactyly, arthropathy, coxa vara deformity and pericarditis) syndrome (n:3), trichorhinophalangeal syndrome (n:1), progressive pseudoromatoid dysplasia (n:2), LIG4 syndrome (n:1), 3M syndrome (n:1), H syndrome (n:1), SPENCD (spondyloenchondrodysplasia, n:3), and nonspecific connective tissue disease (n:8).

Conclusion: Genetic syndromes with musculoskeletal findings are often unrecognized and misdiagnosed as rheumatologic diseases leading to unnecessary procedures and treatments. Summarizing the genetic diagnosis spectrum that can be detected in these patients will increase the awareness of physicians. Results: According to the results of observation, the disease was more common in the age group of 7-11 years (65%), to a lesser extent among children in the group of 12-15 years (35%), less often in the group of 3-7 years (5%). When examining infectious agents, zoonotic infection was detected in 41% (Listeria monozytogenes, Yersinia enterocolitica). Clinical course of nodular erythema in this group was characterized by an expressed activity of the inflammatory process with multiple elements in the lower and upper extremities, joint syndrome, increased ESR to 45± 3.8 mm per hour, CRP 28± 2.5 mg\l. The disease was preceded by an episode of acute infection with an increase in body temperature, intoxication, in some cases with short-term intestinal syndrome, pharyngitis. The rashes were persistent and recurrent, with a slow regression of laboratory activity. Streptococcal etiology of nodular erythema was detected in 37% of cases. There was an increase in ESR to 25±3.8 mm per hour, CRP 15± 2.7 mg/l, a significant increase in antistreptolysin on average 480± 34% IU / ml. with an increase in individual cases to 870 IU/ml. In 13% of cases, erythema nodosum developed after an intestinal infection. Among the pathogens were identified Sh. disenteria, E. coli, Yersinia enterocolitica, enterovirus. The disease was characterized by moderate activity, a good response to etiological therapy and a short course of NSAIDs . An interesting fact was the development of nodular erythema in 4% of cases caused by the Epstein-Barr virus in groups of children from 3 to 7 years and 7-9 years. They had clinic picture with normothermia, no symptoms of intoxication, periodically occurring elements of nodular erythema on the shins, no blood changes. Therapy aimed at eliminating the virus gave a positive result and did not require specific anti-rheumatic therapy. In 5% of cases, the etiology of nodular erythema was not defined.

The clinical course of nodular erythema in children depends on the infectious agent that was the trigger of the pathological process. The higher activity and duration of the disease is caused by zoonotic infection, which requires more active antiinflammatory therapy with corticosteroids, which may be associated with the activation of autoimmunity. This group of children was taken for further observation as a group at risk of developing systemic connective tissue disease. Changes in the etiological structure of nodular erythema and treatment tactics require further study. Introduction: Sjögren syndrome (SS) is a chronic autoimmune disorder characterized by inflammation of the lacrimal and salivary glands leading to oral and ocular dryness. Childhood SS is rare and poorly defined and underdiagnosed owing to the lack of childspecific diagnostic or classification criteria. Objectives: The purpose of this study is to describe 12 cases with pediatric SS in order to better clarify the characteristics of the disease in the pediatric age. Methods: We retrospectively reviewed medical records of patients (pts) with pediatric SS referring to three Italian pediatric rheumatology centers. Due to lack of childhood validated SS-specific criteria, physician diagnosis was the only inclusion criteria. Results: We collected data on 12 pts (9 females). The mean age of disease onset is 10.0 yrs (median 10.2, range 4-17). The mean age of diagnosis is 11.83 (median 11.45, range 6-18). The follow up period varied from 0.1 to 9.3 yrs (mean 3.95, median 5.0). The most common manifestations were articular involvement (mainly with arthralgia) (9/12 pts) and parotid/salivary glands swelling (8/12 pts). Xerostomia and xerophthalmia were found in 6/12 pts and in 4/12 respectively. Vaginal dryness was reported only by one pt. Fever and fatigue occurred in 3/12 and 7/12 pts respectively. We also recorded 3 cases of circulating immune complexes manifestations in 3 pts, purpura (n=2) and glomerulonephritis (n=1). We observed an endocrine involvement in 3 pts (1 metabolic syndrome, 2 autoimmune thyroiditis). Abdominal pain was found in 4/12 pts. All pts were positive for autoantibodies (positivity for ANA or anti-SSA or anti-SSB or FR) at presentation. RF test results were available in 8 pts, all positive. Positive ANA (titer>1/320) and anti-SSA were present in 10/12 pts and in 9/12 respectively. Hypergammaglobulinemia (range 1,6-8.04 g/dl) was found in 8/11 pts (1 NA). Abnormal Schirmer test was observed in the half of cases (6/12). Minor salivary gland biopsy was performed in 10 pts resulting in histological evidence of focal lymphocytic sialadenitis in 9/10. Sonographic evaluation of salivary glands was abnormal in all of the patients (10/10).

With regard to treatment, 6/12 pts received corticosteroids and eight were also treated with one or more DMARDs such a hydroxychloroquine (n=8), methotrexate (n=3), azathioprine (n=1), leflunomide (n=1). Biological therapy was used in 3 patients for systemic involvement: 1 received belimumab and then rituximab, while the other patients received rituximab. Conclusion: Xerostomia and keratoconjunctivitis sicca were not common in our series while recurrent parotid swellings were more frequent than what reported in adults. Pediatric recurrent parotitis should increase the suspicion for Sjögren syndrome. Current diagnostic criteria for SS do not include parotitis and therefore, the incidence of SS may be under-recognized in childhood. The disease is not always benign and patients with severe course may need second line treatment including immunosuppressant and biologics. Introduction: Improving our understanding of pediatric rheumatological (PR) patient population is crucial for pediatric rheumatologists to know rheumatic disease epidemiology and to raise awareness leading to early detection. We didn't find studies of PR disorders presenting in the first year of life. Objectives: The aim of this study is to assess the prevalence of PR disorders with onset in the first year of life. Methods: We retrospectively studied patients observed in our Pediatric Rheumatology Unit between January 1 st of 1987 and December 31 st of 2019. We defined acute (<2 weeks), subacute (≥2 and <6 weeks) and chronic (≥6 weeks). Results: A total of 3751 patients were observed in 32 years. Diseases' onset occurred in the first decade of life in 2290 patients (61%) and in the first year of life in 158 (4,2%). Among the latest group, chronic inflammation was the most frequent group of diagnosis (30%), followed by recurrent inflammation (23%), acute inflammation (11%), infection (9%), infiltrative/ degenerative disorders (8%) and subacute inflammation (3%). The remaining patients (16%) were diagnosed with other disorders classified as miscellaneous. Among chronic inflammation group, 14 patients were diagnosed with juvenile idiopathic arthritis (4 systemic); 14 had neonatal lupus and one patient had polyarteritis nodosa. Among recurrent inflammation group, 13 patients were later diagnosed with PFAPA (periodic fever, aphthous stomatitis, pharyngitis and adenitis), 8 were diagnosed with Behçet disease and 6 had an autoinflammatory disorder. Acute vasculitis was diagnosed in 13 patients (9 Kawasaki disease and 4 acute hemorrhagic edema of infancy). Among infectious diseases group, there were two cases of congenital syphilis with arthritis and two cases of osteomyelitis secondary to BCG vaccination. Conclusion: Rheumatological diseases presenting in the first year of life are not exceptional. Although many patients didn't have a definitive diagnosis at the beginning of the symptoms, many of them were later diagnosed with rheumatic disorders, mostly chronic inflammation (30%), which requires early diagnosis, specific treatment and long-term follow-up. Rheumatic diseases must be considered as differential diagnosis in the first year of life in order to avoid delayed intervention and long term disabilities and sequelae. (1), on the other side measles-induced MAS has rarely been reported (2). Objectives: We present the case of a child known to have sJIA in remission, who presented a Measles primary infection and a secondary KD complicated by MAS. Methods: A 5 years old girl, not fully vaccinated and known to have sJIA in remission under Methotrexate, presented for frequent high grade fever of 3 days duration associated with flat flash red spots on the face and trunk as well as the palms and soles. A Koplik's spot was identified. Conjunctivitis and coryza were also present. Initial viral serology, including measles, returned negative. Fever persisted and on day 7, edema of both hands and feet appeared with bilateral cervical adenopathy, erythematous tonsils, gingivitis, cracked lips and hepatomegaly was noted. All cultures were negative and chest X-ray was normal. Inflammatory markers rose up. Viral serology was repeated and measles IgM came back positive. Cardiac ultrasound ruled out coronary aneurism and the ophthalmic exam showed no uveitis. KD criteria were met and 2g/kg of intravenous immunoglobulins (IVIG) were administered. After 48 hours of clinical improvement, fever reappeared and the patient returned to be ill looking although the rash regressed. We noted high ferritine(2016 ng/ml) together with low C3, decrease in platelets(170 x10 3 /ml) and elevation of hepatic enzymes, LDH and CPK, without increase in the inflammatory biomarkers. MAS was suspected and a bone marrow aspirate showed the presence of mild macrophage hemophagocytosis. Antibodies for Lupus and auto-immune myositis were all negative. Steroids were given, fever disappeared, and spectacular clinical and biological improvements were objected. 2 weeks later, desquamation of all extremities was noted. SARS-COV-2 was not investigated because historically this case presented 1 year earlier than the pandemic. Results: We hereby report, for the first time, KD and MAS triggered by Measles infection in a child with sJIA in remission. The exact mechanism involved in KD-induced MAS and Measles-induced MAS has not yet been defined but a defective immune response is suspected (3). Conclusion: Significant similarities and overlap between measles, KD, sJIA and MAS make an early diagnosis very challenging (1)(3). The recent COVID19 pandemic emphasizes how a viral illness can be responsible of KD and sometimes degenerating in MAS. We report this clinical case as an example of a Systemic Inflammatory Syndrome (SIS) taking place after a viral infection to Measles. In the era of COVID19 pandemic and secondary SIS in children, an additional challenge is present in regions lacking Measles vaccine coverage. 

None declared

The musculoskeletal manifestations of scurvy: a diagnostic challenge for the rheumatologist P2 was a 5-year-old boy, with autism spectrum disorder, malnutrition and severe food selectivity, admitted to our Unit for refusal to bear weight and bruises in lower limbs. The auxological evaluation showed a strongly dystrophic aspect. Coagulation profile and main organ function markers were normal. At nutritional biochemical parameters evaluation, iron and Vitamin C deficiencies were detected (vitamin C: 2 μmol/l). Oral vitamin C therapy was started, with prompt clinical response. P3 was a 7-year-old boy with autism spectrum disorder, admitted to our Unit for lameness and difficulty in walking for a month. At clinical examination, a mottled skin at lower limbs was noted. Joint examination was normal. Auxological parameters and main blood tests were adequate for age. Given the presence of food selectivity, he underwent serum vitamin C dosage (11 μmol/L); hence he started oral vitamin C therapy, with rapid clinical improvement. P4 was a 2 years old boy who was referred for coxalgia and fever. At clinical examination, pale skin, gingival hyperemia, and pain in mobilization of the left hip were present. Microcytic anemia was detected, but main organ and inflammatory markers were normal. No evidence of infection was present. X-ray of femur and knee showed morpho-structural alteration of the distal metaphysis bilaterally. A low intake of fruit and vegetables was reported; hence, dosage of vitamin C was performed, resulting reduced (2.5 μmol/L).

He started Vitamin C oral therapy with clinical response. P5 was a 13-year-old girl with behavioral disorder and intellectual disability, admitted for fever and right knee swelling which appeared two days after a right leg burning. C-reactive protein and ESR were elevated and ultrasound exam confirmed intra-articular knee effusion. Suspecting a septic arthritis, antibiotic therapy was started with laboratory normalization and partial clinical improvement. Considering the persistence of knee swelling after nine days of intravenous antibiotic therapy, the presence of gingival hyperemia and history of food selectivity, vitamin C dosage was practiced (12 μmol/l). Oral vitamin C was administered with complete clinical resolution.

Conclusion: Although scurvy is considered a disease of the past, it still occurs nowadays. Food selectivity associated to autism is a major risk factor for vitamin C deficiency in childhood. Rheumatologists should take into account the diagnosis of scurvy in the diagnostic approach of musculoskeletal disorders in children, especially when development disorders are present. 15.4%), juvenile dermatomyositis (n=1), sarcoidosis (n=1), granulomatous polyangiitis (GPA) (n=1), Sting-associated vasculopathy with onset in infancy (SAVI) (n=1), and oligoarticular JIA (n=1). Respiratory symptoms were present in 6 (46.2%) patients at the time of primary diagnosis. In other patients, the time period between the diagnosis of the rheumatic disease and the onset of the respiratory symptoms ranged from 1 to 12 years. Cough, the most common symptom, was present in 10 (76.9%) patients. Six patients manifested with cough and sputum. Six (46.2%) patients had shortness of breath and one patient had hemoptysis. On the physical examination of one patient, rales and clubbing were detected. High resonance computerized tomography (HRCT) was performed in all patients. HRCT findings were as follows; lymphadenopathy in 8 patients (61.5%), ground glass appearance in 10 patients (76.9%), consolidation in one patient, pleural effusion in one patient, pulmonary nodule in 4 patients (30.8%), fibrosis in one patient, cystic lesions in 3 patients (23.1%), septal thickening in 5 patients (38.5%), bronchiectasis in one patient, and reverse halo sign in one patient. In echocardiographic examination, only one patient had pulmonary hypertension.

Three patients underwent open lung biopsy, and diagnosis was made with pathological examination of the lung tissue. Of these three patients, two (15.4%) had lymphocytic interstitial pneumonia (LIP), and one patient had chronic inflammation and focal fibrosis. Infectious lung disease was not detected in any patient. Ten patients (76.9%) had interstitial lung disease associated with rheumatic disease, one patient had pulmonary hemorrhage, one patient had pulmonary involvement of GPA, one patient had pulmonary involvement of sarcoidosis. There was no statistically significant difference between the first and last spirometry and DLCO values during the follow-up period. Mortality was 7.5% (1/ 13) in this cohort. Active disease was significantly associated with abnormal TC, HDL, and TG levels (p=0.04*), (p=0.03*) and (p=0.04*) respectively. Multivariate analysis of the factors affecting abnormal cholesterol level revealed that SLE is a significant predictor of abnormal cholesterol level . Presence of jSLE increase risk of abnormal cholesterol 9 times more than cases without jSLE. The overall percent predicted was 80%. Active disease is a significant risk factor for abnormal TG with increased risk of abnormal TG by 3.2 among cases with active disease than cases with inactive disease. The overall percent predicted was 75.6%. Conclusion: children with rheumatic diseases showed significant lipid profile abnormalities. Abnormal TC, HDL and TG are significantly associated with active disease. Presence of jSLE increase risk of abnormal cholesterol. Active disease is a significant risk factor for abnormal TG. Therefore, lipid levels should be monitored regularly and managed in patients with paediatric rheumatic diseases to minimize the longterm risk of CVD. Methods: Non-experimental, cross-sectional and descriptive study. A confidential survey was conducted online, aimed at residents and attendings who deal with musculoskeletal pain. Were addressed with the definitions of arthralgia, arthritis, myalgia, allodynia and hyperesthesia (between five to seven options) with only one correct answer. Correct definitions: arthralgia (pain localized to the joint or periarticular structures, as a only manifestation); arthritis (Criterion one or criterion two: 1 -Joint swelling or intra-articular effusion / 2 -Limitation of joint mobilization associated with at least one of the following: a) Pain b) Tenderness c) Swelling d) Heat); myialgia (pain with muscular origin or referred to muscle, regardless of its etiology); allodynia (pain resulting from usually non-painful stimulus); hyperesthesia (coexistence of allodynia plus hyperalgesia -exaggerated responses to tactile and thermal nociceptive and nonnociceptive stimuli 

The association of pure red cell aplasia (PRCA) with thymoma led to the discovery of the autoimmune mechanisms involved in the pathogenesis of this rare disease. Till date many adult case reports have revealed a strong link between PRCA and autoimmune diseases, endocrine disorders, rheumatic diseases, autoinflammation and immune dysregulation. [1] [2] [3] [4] [5] Objectives To stimulate a search for the genetic and immunological roots for a 2.5 years old girl with syndromic face, pure red cell aplasia, type 1 diabetes and polyarthritis. Methods This is a story of a 2.5 years old girl with pure red cell aplasia, type 1 diabetes and polyarthritis. She was normal till 7 months of age. At the age of 8 months, she was diagnosed with type 1 diabetes. She was evaluated by her paediatrician in view of generalized hypotonia, deformed pinna, low set ears, midfacial hypoplasia, blue sclera, umbilical hernia and retracted eyelids. She had multiple episodes of seizures during next few months. To me, she was presented with one year history of polyarthritis with severe pallor requiring frequent blood transfusions. Family history was inconclusive. Musculoskeletal examination showed polyarthritis involving right knee, bilateral ankles, fingers and toes. Further examination revealed haemolytic facies and hepatosplenomegaly. I was not able to make out any facial dysmorphism mentioned earlier by her paediatrician. Results: Table 1 Conclusion Early age of onset, pure red cell aplasia, autoimmune and endocrine manifestations with some doubtful facial dysmorphism inspired me to suspect some known or unknown immune dysregulation syndrome in this child. Genetic analysis would be the best possible option in this scenario if financial condition permits. Introduction: Galactosialidosis (GS) is a rare inherited lysosomal storage disorder (LSD) which is characterized by a defect in the lysosomal glycoprotein catabolism. Here we report, for the first time, a case of a child affected by GS who presented with recurrent episodes of extensive joint inflammation in both knees. Knowledge on GS related inflammatory joint pathology is lacking, which hampers evaluation of possible mechanisms that could give an explanation for the significant arthritic joint abnormalities as observed in our patient.

Objectives: The aim of this study is to describe the clinical presentation as well as the laboratory, radiologic and microscopic features of this extremely rare presentation of GS. Furthermore, we conduct a literature review on LSD's complicated by arthritis in order to evaluate potential mechanisms that could explain the extensive inflammatory joint swelling observed in our patient.

Methods: In this study we present a 12-year-old Turkish boy who was diagnosed with GS (late infantile form) at 17 months of age. From the age of 8 years, the boy presented with episodes of inflammatory joint pathology of the knee. Informed consent was obtained. Alongside the case report, a literature review using Medline was conducted. An extensive list of known LSD's was combined with the terms: "arthritis", "joint inflammation", "synovitis" and "synovial inflammation". Cases in which joint inflammation was based on a probable cause other than the underlying LSD were excluded. Results: In the present case, owing to comprehensive examinations (i.e. laboratory tests, imaging and microscopic examination) multiple possible causes for the recurrent inflammatory joint pathology could be rejected (i.e. no signs of infectious arthritis, reactive arthritis, osteoarthritis, arthritis secondary to a malignancy or crystal induced arthritis). A diagnosis which could explain the clinical picture is the JIA subtype: ANA negative oligo-articular JIA. However, microscopic examination showed numerous foamy macrophages with extensive vacuolization in the synovial tissue of the inflamed joint, which is not associated with JIA. Given the evidence of storage products within the macrophages of the inflamed synovial tissue and no conclusive diagnosis, GS itself should be considered as the primary cause for the recurrent arthritis. An in-depth literature review using Medline for data on inflammatory joint pathology in LSD's showed that 7 LSD subtypes (i.e. Fabry disease, Farber lipogranulomatosis, Gaucher disease type 1, Mucopolysaccharidosis IX, a-Mannosidosis, Fucosidosis and Cystinosis) could present with disease related arthritis. Multiple potential arthritic mechanisms secondary to storage product accumulation in LSD's have been described, such as: dysregulation of innate immunity and increased upregulation of numerous pro-inflammatory proteins. Conclusion: Given the evidence of storage products within macrophages of the inflamed synovial tissue and the absence of other etiological clues, our hypothesis is that GS itself is the primary cause for the inflammatory joint pathology in our patient. Although, GS cannot be linked directly to joint inflammation, lysosomal defects have been associated to pro-inflammatory effects that possibly could result in arthritic disease. Future identification of other patients with GS is required to support the hypothesis of an arthritic clinical phenotype of GS and to assess underlying pathophysiology. Introduction: Joint pain (JP) is a relatively common complaint among children and adolescents. A painful joint in children for many years continues to maintain the status of the most common symptom of juvenile arthritis. However this symptom should not always be interpreted as a manifestation of rheumatic diseases.

Objectives: The aim of current review is to debate of the structure in children with the chief complaint of JP.

Methods: We retrospectively analysed our series of 600 children which attending outpatient department with complaint about pain lasting longer than two months in one or more joints. The clinical, instrumental and laboratory pictures were collected. Special attention was paid to certain aspect of medical complaints, a complete and accurate history and physical examination. Different categories as possible etiologies of JP in children were systematize and detailed.

Results: All children were divided into several groups based on their anatomical and physiological characteristics of osteoarticular system: the first group consisted of 240 children under 6-7 years old, the second group -220 children 7-12 years old, the third group -140 children over 12 years old.

Research suggests that more preschool children were experience bilateral lower extremity pain by "post-walk genesis" due to natural hypermobility, immaturity of sensory innervation of the joints and imbalance of the leg muscles (e.g. growing pains). The second most common cause of JP was associated with intra -or postinfectious factor (viral, streptococcal and chronic focal of infection). The frequency of juvenile arthritis and other rheumatic diseases in children of this age group did not exceed 10%. Special attention was paid to fever, chills, malaise, nightpain and constitutional symptoms with changes in blood lab tests to exclude osteomyelitis (inc specific cause), malignancies manifestation and other bone tumors (less 5%).

The most common causes of joint pain of school-age children were hypermobility syndrome and enthesopathy (primary, secondary). Secondary enthesopathy were result of changes in nutrition, rapid growth and excessive exercise. Also enthesopathy were manifestation of endocrine, gastrointestinal or infectious diseases. The proportion of children with the onset of chronic inflammatory arthropathy also did not exceed 10%. Hypermobility child's syndrome was characterized by harmless pain (inc low back pain), linked to physical activity (less morning stiffness).

Over the past decade, we've seen a gradual increase in the number of children (95% were girls) with knee pain by diagnosed patellofemoral and mediopatellar plica syndromes, patellar tendinitis or idiopathic cause. In most cases children was complicated by syndrome of increased anxiety. The share of true chronic inflammatory arthropathies, including spondylitis, in children of this age group did not exceed 10%. Fibromyalgia were diagnosed less 5%. Introduction: A significant part of patients in rheumatologist's practice is children and teenagers with complaints of pain. The further volume of examination and the choice of treatment course depends on the capability of the rheumatologist to define the inflammatory and non-inflammatory genesis of pain. That makes the problem of differential diagnosis very important.

Objectives: To conduct a comparative analysis of patients with a principal pain complaint to determine if there are significant differences in the groups with the inflammatory and noninflammatory pain genesis. Methods: The retrospective study included children who consulted a rheumatologist in the outpatient clinic in the period 2018-2020 without preliminary selection (n = 176). Of them there were selected children with principal pain complaint (n = 120). According to the diagnosis, the children were divided into 2 groups: those who have inflammatory genesis of pain (A, n =59) and those with noninflammatory genesis of pain (B, n =61). The group A included children with such diagnoses as: reactive, poststreptococcal and juvenile idiopathic arthritides. The group B included children with arthralgia, chronic pain syndrome, orthopedic pathology, fibromyalgia. Results: 1. Groups A and B differ in the average age of the first complaints onset (t-criterion for equality of means) with a high degree of statistical significance (Group A = 7,4 years; Group B = 9,3 years; p = 0.019). Which means that in Group A more often than in Group B first complaints appear in the age between 1 to 10 while in Group B more often than in Group A it happens in the age between 11 to 16. 2. There was a statistically significant difference in the means between Groups A and B in time between the onset of first complaints and the first visit to a rheumatologist (p = 0.03) Also in favor of this conclusion speaks the fact that in Group A the number of visits to a rheumatologist in the same year when the first complaints appear is almost 2 times higher than in Group B. 56% of cases in Group A consulted the rheumatologist the same year when the first complaints appeared in comparison to Group B where only 31% of patients did the same. Below is the table with distribution of cases by the number of years between the first complaints onset and the first visit to a rheumatologist in both groups:

Conclusion: In children with arthritides, the first pain complaints appear at an earlier age (an average of 7.4), and in Group B (an average of 9.3). Patients with arthritis more often visit a rheumatologist earlier (within 1 year after the first complaints) than those with non-inflammatory genesis of pain complaints.

The most common cause of recurrent musculoskeletal pain is growing pain (GP) in children. Differential from rheumatic diseases could be challenging in some cases since there are no diagnostic criteria for GP. Objectives: To analyze GP characteristics in a large cohort of patients in comparison with other non-inflammatory and inflammatory diseases causing limb pain, and to simplify the GP's diagnosis process by using machine learning (ML) techniques.

Methods: This is a multicenter cross-sectional study. Introduction: It is a well-known fact that the period of intensive growth in children is associated with the processes of active bone mass accumulation and coincides with them in time. One of the most distinctive indicators of an increase in the disease incidence among children for the recent decade (+105,3%) can be found in the skeletal disorders resulting from disrupted calcium metabolism and vitamin D deficit. The latter is widespread in Ukraine as it is observed in 92% of schoolers.

Objectives: Establish the specifics of the structural and functional status of the bone tissue in children during the growth spurt, taking account of the degree of vitamin D3 sufficiency.

Methods: The examination covered 147 children aged 9-17 who were divided into three groups depending on the presence of the growth spurt (GS) and its intensity: group 1 -35 children who had become 8-12 cm taller for the year in question; group 2 -32 children who had become taller by 12 cm or more, group 3 -80 children who had experienced no growth spurt. Inclusion criteria were the following: no chronic somatic or endocrine pathologies, no musculoskeletal disorders or mineral homeostasis disruptions; physical exertion corresponding to their age; the children had not been taking any complexes of vitamins and minerals, including vitamin D 3 for 6 months before the examination. Conclusion: Children aged 9-17 showed deficiency of vitamin D 3 reaching 100% which had no correlation with the presence or intensity of the growth spurt. In children who experienced growth spurt, a reduced BMD proved more frequent and correlated with the spurt intensity, however, it did not depend on sufficiency of vitamin D 3. Therefore, during the growth spurt, disrupted mineralization of the bone tissue was influenced not only by the vitamin D deficit but also by the correlation between the bone tissue mineralization rate and intensity of growth in the children. Methods: A self-reported 25 question online survey on QOL of patients with sJIA and AOSD was developed by the non-profit organizations, the Autoinflammatory Alliance, KAISZ/VAISZ, ENCA and sJIA Foundation in English and translated to Dutch. Respondents were recruited by convenience sampling through online social media posts. Data on flares, triggers, family history, and correlation of symptoms with labs were collected in addition to detailed information on QOL during and in-between flares.

Results: Between 2017 and 2019, there were 109 responses; 54 were from parents of children with sJIA, 18 from adults with sJIA, and 37 from adults with AOSD. Interestingly, adults (whether diagnosed with sJIA or AOSD) were more likely to report pain, fatigue, joint swelling or arthritis, nausea & vomiting, and diarrhea during flares than children. Adults were also more likely to describe flares >one month. 80% of patients reported being "greatly" or "severely" limited during flares. Between flares, 20% reported being "greatly" or "severely" limited while 59% were "somewhat" limited. 80% felt their condition affected their studies, job, and career, including 66% of children with sJIA, 100% of adults with sJIA, and 92% with AOSD.

Respondents were asked open-ended questions regarding their experience with disease flares and impact on their lives, and specifically how sJIA and AOSD affected work, career and schooling. Responses regarding the disease experience were classified into 7 theme areas: 1) experience with disease onset and process of diagnosis; 2) health care access, quality, and drug safety concerns; 3) physical impact of the disease including pain and chronic fatigue; 4) social impact of the disease; 5) mental health and emotional impact of the disease; 6) impact on work, career, and employment; and 7) broad impact on life and lifestyle. Responses regarding effect on work, career, and schooling were categorized into 3 theme areas: 1) physical impact negatively influencing school/work productivity; 2) lost work and wages, including unemployment and needing disability benefits, and parents missing work to care for the child; and 3) the socialemotional impact as well as negative effects on mental health. About half of patients regardless of age reported the name sJIA did not represent well the disease, specifically that it did not emphasize the systemic symptoms, and that the disease gets confused with other types of arthritis. Adult patients with sJIA did not like to have juvenile in the name. Conclusion: Children and adults with sJIA and AOSD report high levels of QOL limitation and effect on school, work, and career, both during and between flares. Our qualitative data emphasizes the importance of multidimensional evaluation of disease with ongoing input from the patients, which will provide a foundation for asking more relevant research questions to foster better care and improve QOL. Results: In total 111 participants were included in the study : 62 Juvenile idiopathic arthritis (JIA) patients, and 49 their parents. The mean age of the patients was 13.7 ± 2.3 years. Significant differences were found between patients and healthy children in such HRQoL survey categories like "Autonomy" and "Financial resources" (p < 0.05). Although quality of life in children's with Juvenile idiopathic arthritis was lower than in healthy children in HRQoL survey category "Self perception" (p < 0.05). After analyzing data no significantly differences were found between patients and parents' assessment scores in HRQoL survey categories (p > 0.05). Conclusion: Juvenile idiopathic arthritis has a moderate negative influence on HRQoL survey categories "Self perception", "Autonomy" and "Financial resources" (p < 0.05) according KIDSCREEN-52 queationnaire. Introduction: Juvenile Dermatomyositis (JDM) is often first identified by parents and carers as the red facial rash develops. The rash can progress and lead to young people being misdiagnosed with eczema, scarlet fever or psoriasis. However, over time the obvious signs of JDM can become "invisible" as treatment calms the rashes and masks the outward signs of JDM, until a flare occurs, when the rashes can be a marker for disease activity or progression. As part of a larger study, children around the United Kingdom were asked to discuss their views on whether they wanted people to be able to see their JDM. Objectives: To understand the implications for children and young people from having a disease that has visible and invisible phases and whether they want others to see their JDM, or not. Methods: Children and young people around the United Kingdom who were already consented and enrolled into the UK Juvenile Dermatomyositis Cohort and Biomarker Study were asked to complete a bespoke questionnaire. There was a mix of open and closed questions, and it was administered in paper format to all children and young people between the ages of 8 and 19 years of age for self-completion, either on the paper forms, or via a secure web-based software system. The questionnaires were administered at the end of 2018. Numeric data were described and qualitative data were analysed using standard content analysis. Results: 246 questionnaire packs were sent out, with 127 (52%) being returned. Of these 4 could not be used due to practical reasons, such as only demographic data being completed, which left a sample of 123. 98 (80%) of the 122 who responded said other people could not see their JDM, with only 11 (9%) saying it was visible and 13 (11%) saying they did not know if others can see it. 1 did not respond to the question as said their JDM has gone away. They were then asked whether it was a good or bad thing for others to be able to see their JDM or for others not to be able to see it. 41 young people left comments as to why it was a good thing, 36 left comments as to why it was a bad thing and 14 left comments to why they said don't know, table 1 presents the top ranking response for the three multichoice answers. Conclusion: This study has highlighted the disparity between young people wanting others to see their JDM so that they gain more understanding and empathy from those around them, but equally, wanting their JDM to be invisible, so that they feel the same as their peers. Whilst many paediatric rheumatic conditions are in fact invisible, our data illustrate that JDM often gives children and young people a taste of both visible and invisible phases of disease activity. As one young person said "It's not good, nor badit's good that it's invisible sometimes so I can blend in without the disabled stereotype. However, sometimes it needs to be seen so I can be understood and not challenged".

Disclosure of Interest: None declared Multidimensional Assessment Report (J-FiMAR) which includes comprehensive patient self-report questionnaire and numerical rating scales to measure pain, fatigue, headache, sleep quality, physical function, psychological state, health-related quality of life, satisfaction with illness course. The J-FiMAR has been devised according to the Outcome measure in Rheumatology (OMERACT) guidelines. Discriminant ability of the multidimensional tool was evaluated by testing it in a control group including healthy controls and patients affected by active juvenile idiopathic arthritis (JIA). The psychosocial consequences of chronic pain were evaluated by using the Children Depression Index (CDI) and the Multidimensional Anxiety Scale for Children (MASC). The objective sleep quality was measured by overnight polysomnography. Results: Table 1 shows characteristics and the most represented somatic symptoms in our cohort of JFS patients at the study enter. Polysomnography was performed in 21 patients with sleep disturbance; 8/21 (38.1%) showed an electroencephalographic pattern of alpha wave intrusion in slow wave sleep (SWS). The presence of objective sleep disorders was significantly correlated to CDI score rs -0,775 (p≤0,0001) and MASC 0,61 (p=0,005). From November 2016 to April 2020 J-FiMAR was completed by 43 JFS patients (F 35 (81.4%), median age 14.7 years [7.1-17.6], median disease duration 1.9 years [0.1-7.8]) in 125 visits. All patients filled out the questionnaire in a short time (<15 minutes) and considered it simple and easy to understand. JFS patients showed significantly higher score for pain, fatigue, poor physical function and measure of psychological distress than healthy controls and JIA patients (p<0.05 for each item). Conclusion: JFS patients presented significantly higher pain experience, functional disability, and impaired quality of life than patients with active JIA. A relevant percentage of JFS patients experience sleep disturbances, which were correlated with mood and anxiety disorders. Our multidimensional tool was feasible and able to quantify global JFS severity. This multidimensional tool, by measuring the main domains affected by the disease, could be promising to individualize treatment strategy and to test its efficacy.

Disclosure of Interest: None declared Introduction: Fatigue is a subjective state of overwhelming, sustained exhaustion and decreased physical and mental capacity, which is not relieved by rest. Fatigue is the most common complaint in children and teens with an autoinflammatory disease, besides the disease related flares. The purpose for this study was to show that fatigue is a serious issue for children and young people with autoinflammatory diseases. We hypothesized that age, gender and/ or the type of autoinflammatory disease could have differing effects on the fatigue experience. Objectives: We aimed to investigate fatigue in children and young people (CYP) with autoinflammatory disease, including how this affected them on a daily basis. Methods: CYP with autoinflammatory diseases were invited to complete an online survey, providing details about their fatigue and how it affected them. The survey was developed by the non-profit organizations Autoinflammatory Alliance and KAISZ/VAISZ, in English. Respondents were recruited by convenience sampling through online social media posts. Data on age, gender and disease were collected in addition to information on their experience of fatigue on school and social interaction. A total of 114 CYP (age range 7-18 years) with an autoinflammatory disease responded to the survey (52% female). Results: The majority of respondents (81%) reported experiencing both mental and physical fatigue. Respondents were asked how much their fatigue affected them, on a scale of 0 to 10; overall, the mean fatigue score was 6.6. However, young people aged 15 or over reported a significantly higher impact than those aged 11-14 years (mean 7.5, p=0.012). Different autoinflammatory diseases were surveyed: CRMO 25%, CAPS 20%, PFAPA 12% also Unclassified SAID (uSAID) with 23%.

In the open-response portion of the survey, 81% of respondents reported that fatigue was physical, as well as mental, in their experience. Most (89%) reported that someone had doubted their fatigue in the past; 29% had found their teachers had doubted them, 26% had friends who doubted them, and 24% reported that they felt their doctors had doubted them. Children and young people also felt a number of activities made their fatigue worse (table 1) ." Conclusion: CYP with autoinflammatory diseases experience physical and mental fatigue. Health professionals and teachers should listen to patients reporting fatigue, validate their experience, and help find ways to support them. Identifying resources to help the patients with fatigue, and referrals to therapy and mental health resources as needed may help the patients to better cope and manage their chronic disease. Further studies will include patient engagement in designing questionnaires about all aspects of life and autoinflammatory disease will help our understanding of these complex conditions and how they affect patients. Introduction: Scleromyositis is the most common overlap syndrome but is rarely observed in childhood. This disorder involves two different autoimmune diseases: systemic scleroderma (SSc) and polymyositis (PM). Objectives: To describe the clinical course of a SSc/PM syndrome in a young girl. Methods: Case report Results: An 11-year-old female was admitted to the Neurological Unit of our hospital for creatine phosphokinase (CPK) increase and hypertransaminasemia associated to sporadic episodes of right calf pain. Familiarity for muscular dystrophy was reported in the maternal branch. Muscle tone and trophism were preserved at initial neurological evaluation. Laboratory investigation confirmed increased muscle enzyme levels, including CPK (x70) (CK-MM 94.5%, CK-MB 5.5%), aldolase (x7), cardiac troponin (x10) and myoglobin (x10). Suspecting a primary muscle disease, she underwent a total body STIR-MRI which showed a diffuse edema of gluteus medius bilaterally and a muscle biopsy revealing a marked muscle damage with dystrophic aspects and normality of the tested proteins. A genetic extended panel for congenital myopathies resulted negative. After 4 months, a new clinical examination showed the occurrence of general skin induration, sclerodactyly and tightening of the face skin.

Appearance of dysphagia was also reported, and muscle enzyme increase persisted. In suspicion of an SSc/PM overlap syndrome, she was referred to our Unit. Nailfold capillaroscopy showed capillary dilatation and branching, megacapillaries and diffuse microhemorrhages. Reduction of esophageal contractions amplitude and hypotensive lower esophageal sphincter pressure were observed at esophageal manometry test. High-resolution CT of lungs and pulmonary function testing were normal. Skin biopsy showed sclerodermiform findings. Immunological studies revealed a positivity of antinuclear antibody (1:320) and anti-Ku. Anti-PM-Scl resulted negative. An oral corticosteroid therapy (prednisone, 1.5 mg/kg/day) was started in association with subcutaneous Methotrexate (15 mg/m 2 /week) and intravenous immunoglobulins (IVIG) (2gr/kg every two weeks). Improvement of skin manifestation, joint mobility, as well as normalization of serum CPK levels were observed. Over 3 months, prednisone and IVIG were slowly discontinued up to the ongoing dosage of 0.9/mg/day and 2 gr/kg every 4 weeks, respectively. MTX is still ongoing at the same dosage.

Conclusion: The diagnosis of overlap connective tissue disease syndromes may be challenging in pediatric age. Different symptoms may be prevalent at different stages throughout the course of the disease. In our patient, a localized myositis preceded the SS onset by about four months. Even though the use of high dose of corticosteroids is associated to a higher incidence of renal crisis in patients with cSS, a combined therapy with high doses of oral steroids, IVIG and MTX was safe and effective in skin, muscle and joint symptoms in our patient. Results: A total of 336 images were obtained from 118 healthy children included in the study and 708 capillary measurements were made. Capillary density was significantly higher in Group 4 than in Groups 1 and 2. Arterial width was significantly lower in Group 1 as compared to Group 3 and 4, and in Group 2 as compared to Group 4. Apical loop width and capillary distance were significantly lower in Group 1 compared to Group 2 and 3 and 4. There was no significant difference between the age groups in terms of capillary length and venous width. There was no difference between the groups in terms of capillary morphology. In total 336 image evaluations, capillary tortuosity was <50% in 67.8%, and > 50% in 4.2%, and capillary crossing were <50% in 52.5% and> 50% in 3.4%. While the enlarged capillary was 4.7% and the avascular area was 4.2%, capillary branching, capillary meandering, microhemorrhage, and giant capillary were not detected in any case. There was a good level of agreement between the researchers, as 20 cases with 120 capillaries were evaluated with a good level of agreement (Table 1) .

Conclusion: This is the first study to evaluate capillary morphology in healthy Turkish children. This study also adds that some special forms such as enlarged capillary and avascular area, which is always named as pathological in adult age, can be seen in healthy children. These data will be guiding in capillaroscopic studies in various patient groups, particularly in children with collagen vascular diseases. Methods: 308 patients with JIA were tested for HLA-B27. They were divided into 2 groups: 1) HLA-B27 positive and 2) HLA-B27 negative.

Results: 100 patients (32,5%) were HLA-B27 positive and all of them are fulfilled the EULAR criteria of entesitis-related arthritis (ERA). The group 2 consists of 208 patients (67.5%). There's no statistical difference between both groups in active joint count, ANA-positivity and uveitis frequency, the rate of use methotrexate and time before biologics. No difference in axial cervical spine 12 (12.0%) vs 21 (10.1%) (p=0.613) and sacroiliac joints 18 (18.0%) vs 23/207 (11.1%) (p=0.097) involvement was observed. HLA B27(+) patients often received pulse therapy with methylprednisolone due to increased inflammatory activity and severe arthritis (22% vs 11.1%, p=0.011). Other parameters are listed in Table1.

Conclusion: patients with HLA-B27 positivity were characterized by male predominance, more often hip involvement, higher laboratory activity and the need for more frequent use biologics. The rate of axial involvement wasn't different in HLA-B27 positive and negative patients, that needs further study and creating more accurate classification criteria for JSA.

Disclosure of Interest: None declared Introduction: Although gut is increasingly recognized as origin and/or target of inflammation in adult onset spondyloarthritis (SpA), the incidence of gut involvement in juvenile SpA (jSpA) patients is still largely unknown, mostly due to the lack of reliable non-invasive tests.

Objectives: We performed a cross-sectional study of fecal calprotectin (fCAL), a surrogate marker of gut inflammation, in patients with jSpA, other forms of juvenile idiopathic arthritis (JIA) and noninflammatory (NI) conditions. Methods: fCAL was measured by commercially available assay in stool samples of enthesitis related (ErA), psoriatic (PsA) and patients with other JIA subtypes (oligo-and poly-articular) who fulfilled ILAR criteria, as well as in children with NI causes of musculoskeletal pain (NI-MSD), regardless of the gastrointestinal (GI) symptoms (Table 1 ). fCAL was compared among different groups of patients and correlated with demographic data, clinical characteristics, treatment modalities and disease activity measured by jSpADA. The values were also dichotomized to <50 mg/kg, 50-200 mg/kg, and >200 mg/kg, which was regarded as normal, slightly increased and increased, respectively. Ileocolonoscopy was performed in one patient. 

Our study has shown that fCAL levels are significantly higher in ErA patients compared to other JIA (p=0.03) and/or NI-MSD (p=0.03) patients. Moreover, almost a third of patients with ErA had levels of fCAL above the range regarded as normal, which adds to the number of evidences for a gut inflammation in this particular type of JIA. Besides, the fCAL levels were higher in those with axial involvement, which further suppots the association of gut and axial inflammation in children with ErA. Although endoscopy remains a gold standard for the diagnosis of gut inflammation, fCAL can help to select children with ErA who might benefit from this invasive procedure, regardless of the GI symptoms, as shown in one patient with the highest fCAL concentration in our study. Moreover, fCAL levels seems not to be influenced by disease characteristic and/or concomitant therapy intake. Therefore, fCAL should be a part of diagnostic workup in children with any type of JIA, but most importantly in those with ErA. evaluate potential predictor variables of Magnetic Resonance Imaging (MRI) SIJ remission Methods: Retrospective review of prospectively collected data. We included patients with ERA (according to ILAR criteria) continuously treated with anti-TNF agents for ≥ 12 months who had at least two MRIs of the sacroiliac joints performed before starting anti-TNF therapy (baseline) and during the follow up ( > 12 months after anti-TNF treatment). SI joints were examined using T1-weighted images, T2 fastsuppressed and short-tau inversión recovery. The SPARCC-SIS was scored by two pediatric radiologists. SPARCC-SIS assessed the presence, depth and intensity of bone marrow edema (BME) on consecutive six slices in the iliac and sacrum bones . Scoring is composed by: BME (0-48), BM intensity (0-12), BM depth (0-12 Introduction: Several paediatric patients manifest conditions commonly misdiagnosed as spider bites, which however, can include other arthropods bites; bacterial, viral, and mycotic infections; vasculitis; dermatological diseases; miscellaneous conditions as drug reactions, chemical injuries. Objectives: In Italy, spiders which are likely to be associated with severe toxin mediated tissue damage are uncommon, especially in urban zones. However, a minor trauma may be a precipitating factor for pyoderma gangrenosum particularly over the legs, in association with inflammatory bowel disease, haematologic diseases and Juvenile Idiopathic Arthritis (JIA). Methods: We describe a 11-years old boy with pyoderma gangrenosum complicated spider bite in association with systemic JIA (sJIA). The patient was in clinical remission after the start of the sJIA, occurred two months before, still treated with tapering doses of steroids and canakinumab, with the normalization of inflammatory parameters (CRP, ESR, SAA, ferritin) and clinical manifestations. Only a mild arthritis of the knee persisted and for this reason he was still treated with steroids. Furthermore, he developed hyperglycemia, requiring insulin treatment. The first dermatological manifestation which he referred was a red dot of the leg skin. In a few days, the erythema enlarged, involving an area of 7 x 7 cm, with oedema, pain, and blisters, evolving in a necrotic lesion, with purulent exudate, surrounded by a haemorrhagic zone. Results: Haematological controls revealed neutrophilic leucocytosis, increased CRP and procalcitonin. He started treatment with intra venous administration of teicoplanin plus ceftriaxone, with no resolution of the clinical manifestations and the reduction of leukocytosis, CRP, procalcitonin. A culture swab was performed and was positive for Pseudomonas Aeruginosa, confirmed by PCR on the culture. He started ciprofloxacin and surgical curettage of the lesion, with the resolution of the lesion and the normalization of biochemical parameters.

Conclusion: The aspect of the lesion and its evolution were evocative of a spider bite suggested by anamnestic records, complicated by a pyoderma gangrenosum secondary to Pseudomonas Aeruginosa. The underlying disease, the immune suppressive treatment, with steroids and biological drugs, the hyperglycaemic pattern of the patient allowed the severe evolution of the spider bite. Children in treatment with immune suppressive and/or biologic drugs are at high risk of infections. Skin lesions, as arthropods bites, can be a facility for superinfection, with possible haematological and systemic diffusion.

The strict application of the ILAR 1 requirement for the presence of documented arthritis for the diagnosis of sJIA, early in the disease course, may result in unnecessary delays in initiating appropriate treatment. In preliminary PRINTO 2 classification criteria for sJIA, this mandatory requirement of documented arthritis has been modified.

To measure performance of preliminary PRINTO classification criteria for sJIA in our Indian cohort. Methods I gathered a data of seven sJIA patients who attended dev children's hospital between Jan 2019 and Jan 2020. My data included demographics,clinical presentation, laboratory parameters and outcome of these patients. All these patients were diagnosed at an early stage by clinical judgement irrespective of fulfilment of ILAR criteria. I applied preliminary PRINTO classification criteria for all.

Average age of selected children (4 girls and 3 boys) was 5.1 years. Conclusion A preliminary PRINTO classification criteria for sJIA has been validated in our cohort. There are many raised inflammatory markers in most of these patients other than WBC count. These markers should be considered to be added in supportive laboratory criteria to be more specific towards the diagnosis. It is important to add PID in exclusion list especially in a case of sJIA with MAS at onset. 3 Trial registration identifying number Leningrad's Regional Children' Two patient stopped treatment within 6 months from therapy start: due to primary inefficiency (1) and allergic reaction (1). Five (5/11) patients were-co-administered with cDMARDs, other 5with oral GC, and 6 subjects had been previously exposed to other biologic drugs. Whole 5 patients stopped therapy due to secondary inefficiency: 2 patients were switched on TOC, other children were switched on ETA (1) Introduction: Systemic juvenile idiopathic arthritis (sJIA) is a rare, complex auto-inflammatory disease with significant morbidity including fever, rash, serositis and articular problems. With the availability of interleukin-1 (IL-1) and IL-6 inhibitor treatment, morbidity has significantly reduced and the outcome for sJIA patients has improved. However, differences in access to care and differences in treatment strategies between countries in and outside of Europe remain a concern.

Objectives: The Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) consortium aimed to develop best practices for paediatric rheumatic diseases in order to decrease differences in care between European countries. Here, we present the final results of the literature review and a series of consensus meetings on defining overarching, diagnostic and therapeutic recommendations for diagnosis and treatment of sJIA.

Methods: The SHARE methodology has been previously published, including the use of the EULAR standardized operating procedure for developing best practice recommendations. As per these guidelines, a methodologist provided supervision during the process and consensus meetings. Conclusion: HScore seems to perform slightly better than MS-score for the diagnosis of MAS in our cohort. 

Early inhibition of IL-1 is discussed to play an important role in the disease course of sJIA 1 . Assuming that pretreatment with other DMARDs leads to a later start of therapy with canakinumab, this analysis evaluates the effectiveness of canakinumab as first-line vs. second-line DMARD.

To evaluate the effectiveness of canakinumab as first used biological DMARD in sJIA compared to canakinumab in sJIA-patients pretreated with other DMARDs. Methods SJIA-patients documented in the German Biologic Registry for Pediatric Rheumatology (BiKeR), who were exposed to canakinumab, were identified. For the first-line (FL) group DMARD naïve patients were selected, prior treatment with corticosteroids and/or NSAIDs was allowed. Patients receiving any DMARD prior to canakinumab entered the second-line (SL) group. Both groups were compared in a retrospective intention-to-treat-analysis. Effectiveness 

Canakinumab treatment showed good effectiveness in sJIA both as first-and second-line DMARD. After 6 months the use of canakinumab as first-line DMARD is associated with higher response rates compared to second-line use. Our data support the hypothesis that early treatment with canakinumab is associated with good therapeutical response and a positive effect on the disease course of sJIA. Results: A total of 11 children (9 girls) with SLE were identified. Median age of symptom onset and diagnosis was 14 years(range 8-17 years) and 11 years respectively. The presenting manifestations were fever(5), oral ulcers(3), alopecia(3), malar rash(4), photosensitivity(5), renal involvement (5), seizures(1) and gastrointestinal complaints (1) apart from some unusual manifestations of isolated peripheral arthritis(1), isolated bilateral pleural effusion(1), macrophage activation syndrome(2). Laboratory investigations: Hemogram revealed anemia in 8 children and thrombocytopenia in 5. Urine examination showed nephrotic range proteinuria in 1 child and subnephrotic proteinuria in 2.

Microscopic hematuria was noted in 2 pateints. Renal function tests were deranged in 2 cases. ANA, Anti dsDNA positivity and hypocomplimentemia were present in all. Renal biopsy was done in 4 patients, 2 had class IV, one class III and one had class V lupus nephritis. All patients were initiated on hydroxychloroquine and photoprotection. Children with renal involvement were given pulse methylprednisolone followed by tapering doses of oral prednisolone and intravenous, monthly cyclophosphamide. Azathioprine was used as maintenance therapy in all. Subcutaneous weekly methotrexate was used in 2 patients. One child (MAS) died during disease course. Disease continues to be in remission in rest. Conclusion: We found a significant female preponderance in our study group. Renal involvement was the commonest presentation. Some unusual presentations were also seen. Early recognition of SLE is critical for timely initiation of appropriate treatment. This is the first report of a cohort of Pediatric SLE from this part of India. Introduction: Autoantibodies in AHAI may be IgG/IgM/IgA. AHAI can be divided into primary or secondary (e.g. SLE, lymphoproliferative diseases, infections, medications). It is also classified based on the temperature at which the antibody reacts to erythrocytes, and can be warm (IgG or IgA) or cold (IgM or C3). In warm AHAI, the antibodies react at temperatures ≥37ºC, not activating the complement system and not undergoing agglutination in vitro. In cold AHAI, antibodies react at temperatures below 37ºC, activating the complement system with in vitro agglutination.Mixed AIHA (warm and cold) is rare and occurs in <10% of AIHA cases and can occur at any age, but is extremely rare in children. The prevalence of the mixed form is less than 1/1,000,000 patients with AHAI.

Objectives: To report a rare case of mixed AHAI and idiopathic intracranial hypertension(IIH) in a 15-years old female patient with a previous diagnosis of SLE and APS. Methods: Case report and literature review.

Results: A 15-years old female adolescent previously diagnosed with SLE/APS since 2017 was in remission on hydroxychloroquine(400mg);azathioprine(150mg);aspirin(100mg);vitaminD3(1.000IU);calcium(1g), and sunscreen. In April 2020 , she had a relapse presenting with fatigue, myositis, headache, hypocomplementemia, and severe autoimmune hemolytic anemia (Hb of 4g/dL) (SLEDAI-2K=18 points). Mixed AHAI was diagnosed base on a Direct/Indirect Coombs test 4/4+;DirectAntiglobulinTesting showing anti-IgA(weak),anti-IgM(3+/4+),anti-IgG(3+/4+),anti-C3c(weak),anti-C3d (3+/4+);IgG1/3subclasses with a reaction of 1:100(2+/4+);an eleven cell antibody panel positive revealing a cold and warm antibody, and adsorption technique revealing a cold and warm autoantibody. Chest CT showed bibasilar subsegmental atelectasis, head CT/MRI was normal and LP showed a high opening pressure of 45cmH2O with a normal cell count. After the procedure, the patient reported improvement in the pain and was diagnosed with IIH. The patient was screened for secondary causes for AHAI (table 1) due to the unusual mixed type pattern and serology was positive for Chlamydia trachomatis (IgM) and Mycoplasma pneumoniae (indeterminate-IgM/positive-IgG) suggesting a recent infectious trigger causing reactivation of the underlying disease with a probable cross-reactivity. The patient treated with 10-days of clarithromycin. Before the infectious screening came back negative, AHAI was treated with a single dose of IVIG(1g/kg) and then, with 3-days of methylprednisolone(1g/day). Azathioprine was replaced by mycophenolate mofetil. Due to headache recurrence, acetazolamide(500mg/day) was started, and the patient referred no pain. The patient was discharged with a resolution of the symptoms. Objectives: To our knowledge, the association of GBS and BBE has been described in adults only. Methods: We here describe a child presenting at SLE disease-onset with an overlap of peripheral (GBS) and central (BBE) nervous system manifestations, highlighting the possible association between these two entities in children.

Results: An 11-year-old healthy girl presented with acute ataxia, ophtalmoparesis and altered level of consciousness, rapidly followed by areflexia, facial paresis, swallowing difficulties, sensory deficits, paresis in all four limbs and respiratory insufficiency. These symptoms were accompanied by pleuro-pericardial serositis, proteinuria and hypertension. Immunological investigations revealed the presence of positive ANA and ds-DNA antibodies. The renal biopsy showed a stage III lupus nephritis. Hence, the clinical, laboratory findings and biopsy report led to the diagnosis of pSLE. Brain and spine MRI did not show any abnormalities; diffuse slowing compatible with nonspecific encephalopathy was seen on EEG. Nerve conduction studies (NCS) confirmed the clinical suspicion of acute polyradiculoneuropathy with proximal interruption of motor nerve conduction, compatible with Guillain-Barré-like syndrome. CSF analysis (performed twice) remained normal. The patient was treated with glucocorticoids, intravenous immunoglobulins, cyclophosphamide as well as plasmapheresis. The neurological and physical symptoms improved gradually with complete neurological recovery four months after onset. Conclusion: Overlapping forms of BBE/GBS have never been described in association to SLE in children. Our patient's presentation and evolution fulfilled the criteria for such an overlap, occurring at pSLE onset. Although SLE and BBE/GBS are rare entities, our case suggests that there may be a common underlying immune background. This association should be recognized early for rapid and appropriate treatment initiation.

Infantile antiphospholipid antibody syndrome: acquired and de novo APL appearance in four infants T. Giani 1 , G. Ferrara 2 , A. Mauro 3 , R. Cimaz 4

Introduction: Antiphospholipid syndrome (APS) is a rare condition in the neonatal age. In most cases it is considered a passively acquired autoimmune disease, due to a transplacental passage of maternal antiphospholipid antibodies (aPL). Exceedingly unusual is the de novo production of aPL in newborns and infants. Objectives: To describe four infants who developed an early brain stroke with increased and persistent levels of aPL, even after six months of life. Methods: We reviewed the clinical charts of four such infants, followed from diagnosis up to two years after the disappearance of aPL. Conclusion: Common characteristics of these four children are the development of brain stroke and the increased and persistent aPL levels even after six months of life. This opens the window on a gray zone related to the origin of these antibodies (maternal or neonatal) and on their role in the pathogenesis of the infantile brain stroke. patients had over 20% of their monitoring completed but only 2 had over 80%. Aspects of monitoring that were more time intensive or were required less regularly were most frequently overlooked. There was a statistically significant increase in the percentage of completed monitoring in those patients for whom the Lupus Checklist was used compared to patients where a checklist was not used (p=0.00).

Conclusion: There is significant room for improvement in the monitoring of these patients with JSLE in the rheumatology clinic. This audit illustrates that more diligent use of the Lupus Checklist and an overall improvement in sustained use of the checklist will help to improve monitoring of these patients. Evidence suggests that checklists are underutilised in medicine and wider implementation of this simple tool could improve patient outcomes. 3, 4, 5 Interventions such as in person or electronic reminders, or audits with feedback to physicians could improve usage over time. The application of the Lupus Checklist or a similar document in other paediatric clinics is important for comprehensive monitoring of a condition as complex as JSLE and has the potential to prevent ongoing damage and medication toxicity in this high-risk population.

juvenile onset and this cluster have may more severe kidney, neuropsychiatric or hematological involvement.

Objectives: The aim of this study was to assess the clinical and laboratory characteristics, disease activity, and treatment response of patients with juvenile SLE (jSLE).

Methods: This is a retrospective study involving patients between 1 July 2016 and 1 January 2020. The data of patients diagnosed with jSLE and followed up for a minimum of 6 months, were collected. The SLEDAI-2K scores at initiation and at the follow-up (1st, 3rd, 6th, and 12th months of treatment) were examined. The SLEDAI-2K score was considered to be ≤4, for disease remission status.

Results: A total of 49 children were included in to the study. The female/male ratio was 4.4/1 and the median age of the patients at the diagnosis was 13 (IQR: 11.1-15.2) years. The median follow-up of patients was 19 (IQR: 12-25) month. Four of the patients were diagnosed with monogenic SLE. Two siblings were diagnosed with c3 deficiency and two were diagnosed with familial chilblain lupus. The most common clinical findings were found musculoskeletal complaints (69.4%), malar rash (51%), oral ulcers (38.8%), and fever (30.6%), respectively in over all the group. The frequency of involvement of the system and organs was as follows; mucocutaneous 77.6%, musculoskeletal 69.4%, renal 44.9%, hematological 34.7%, serous membranes 16.3%, neuropsychiatric 12.2%, respectively. All patients had anti-nuclear antibody positivity, while 46.9% had anti-ds DNA, 14.3% had anti-Sm and 8.2% had antiphospholipid antibody positivity. While all patients received hydroxychloroquine treatment, 22.4% of the patients were received were mycophenolate mofetil, 22.4% were azathioprine, 14.3% cyclophosphamide, 12.2% methotrexate and 10.2% were rituximab. The median SLEDAI-2K score was 14 (IQR: 10-18.5) at admission, besides it was found to 6 (IQR: 4-12), 4 (IQR: 2-6), 2 (IQR: 0-6) in the 1st, 6th and 12th months of treatment, respectively. While 98% of the patients had active disease at admission, 67.3% at 1 months, 32.7% at 6 months and 22.4% at 12 months still had active disease (SLEDAI-2K >4). Patients with initially high SLEDAI-2K scores had significantly lower remission rates in the first month (p=0.003). It was observed that patients with high SLEDAI-2K scores in admission were more resistant to conventional immunosuppressive treatments and the use of rituximab was more frequent in these patients. At least one major organ (renal, hematological, neurological) were affected in 57% of patients. The remission rate of these patients at 6 months was found significantly decreased compared to the others (p <0.005). Renal biopsy was performed in 21 patients (42.9%). 12 of them had type 4 lupus nephritis (LN), 5 had type 2, 2 had type 3, and 1 had type 5. It was observed that patients with renal involvement were the group that reached remission latest. Conclusion: The presence of high initial SLEDAI-2K scores and the major organ involvement have poor predictive value to achieve inactive disease. A two year old girl of consanguineous parents presented to hospital at 13 months of age with fever and erythematous macular rash on her cheeks which spread to her nose, chin, and ears. The rash started a month prior, and progressed over her entire body. A skin swab grew staphylococcus aureus but the rash didn't respond to topical antibiotics. Review of systems was unremarkable except for longstanding oral thrush and diaper rash. Birth and family history were unremarkable. On exam she had a diffuse, erythematous, morbilliform eruption over her face and body. She had facial swelling, orbital edema and vasculitic oral ulcers. She had leukopenia mainly neutropenia, low hemoglobin, with normal platelets. Her liver enzymes and erythrocyte sedimentation rate (ESR) were high while C-reactive protein, immunoglobulins, C3 and C4 were normal. Cultures were negative, however she was positive for adenovirus, mycoplasma and EBV (EBV load was 6000 IU/ml ). Autoimmune hepatitis work up was negative. The direct coombs test, antinuclear antibodies (1:640), Ro, RNP and SmD were positive. CH50 came low as well as C1q level of 4 mg/dL (normal range 12-22 mg/dL). Lymphocyte subsets showed reduced CD4 and NK cells. Bone marrow aspiration showed active marrow. Skin biopsy showed chronic non-specific inflammation (immunofluorescence and electron microscopy were not available). Echocardiogram showed dilatation of the left coronaries. She was treated with intravenous immunoglobulin (IVIG) for Kawasaki disease with no improvement. Therefore pulse steroid 30mg/kg followed by 2 mg/kg was initiated. Her rash, facial swelling and abnormal blood counts improved dramatically. Whole Exome sequence showed homozygous variant c.469G>T p.G157C at the C1qA gene. While tapering steroids she flared so subcutaneous methotrexate was started. Unfortunately, she continued to have rash, leukopenia and high liver enzymes, so treatment was switched to mycophenolate mofetil and hydroxychloroquine. However she did not improve and started to have recurrent bacterial and viral infections that included cellulitis, gastroenteritis and upper respiratory tract infection. We started her on regular IVIG, which helped with infections and allowed for weaning of steroids. However she developed alopecia and lower limb spasticity with delayed walking. MRI brain and spine was normal. Upon reanalysis of the WES, two other homozygous mutations at KIF1C and APG7 were identified and associated with spastic paraplegia, but reported as variants of unknown significant. Fresh frozen plasma (FFP) transfusions were started, initially weekly, then every two weeks and subsequently every four weeks. The rash disappeared, leukopenia and ESR improved and we were able to discontinue steroids Conclusion: Early-onset SLE with a severe course of disease raises the possibility of a genetic etiology. We are reporting, for the first time, a rare missense mutation G>T in exon 3 of the C1qA gene that resulted in an amino acid substitution that is pathogenic. Interestingly, she had other mutations associated with neurological manifestation that never reported together before and altered her phenotype. She has responded well to FFP as has been reported in a few case reports Results: A total of 148 pSLE under the age of 13 years were included, 30% (n = 44) were males. The overall mean age at diagnosis was 7.6 ± 3.5 years and median disease duration was 9.5 (5-13) years. HUV was diagnosed in 34.5% (n = 51) of pSLE cohort. pSLE with UV were more likely to be males (57% vs 15%; P < 0.001), diagnosed at a younger age (5.9 vs 8.5 years; P < 0.001), have a family history of SLE (53% vs 36%; P = 0.044) and have conjunctivitis more frequently (32% vs 5.3%; P < 0.001) than pSLE without UV. pSLE with UV were also less likely to have CNS involvement (7.6% vs 20%; P = 0.045) and hematological manifestations such as leukopenia (9.4% vs 24%; P = 0.028) and thrombocytopenia (5.7% vs 18%; P = 0.045). In addition, pSLE with UV were more likely to be associated with low C3 complement count (94% vs 66%; P < 0.001) and positive cytoplasmic ANCA (11% vs 0%; P = 0.022).However, the pSLE with UV cohort were less likely to be associated with ANA (65% vs 83%; P = 0.016), DsDNA (56% vs 72%; P = 0.042) and perinuclear anti-neutrophil cytoplasmic antibodies (33% vs 55%; P = 0.047). Conclusion: We report a high occurrence of HUV in pSLE cohort (34.5%) associated with unique demographic, clinical features and laboratory features. The debate regarding whether HUV is a rare subset or unusual type of SLE, or is a separate entity altogether, continues. However, the overlap in clinical, laboratory and genetic mutation supports the notion that HUV and SLE fall into the same spectrum of autoimmune disease with similar disease pathogenesis. However, further studies are needed to reach clear conclusions regarding the relationship between HUV and SLE. Introduction: The last decade has brought a lot to the approaches to the diagnosis and treatment of juvenile arthritis. In Russia, the actualization of the problem of diagnosis and treatment of JIA required the development of federal standards, which provide the most detailed algorithms for medical care, both at the stage of inpatient and outpatient care. In the regions of the Russian Federation, the effective use of these documents required a whole range of additional educated activities, both with students of medical universities, as well as with the medical and nursing community, in addition, a set of work was carried out to create a regional regulatory framework. In the total biological therapy pool, 67% of patients receive TNF-alpha inhibitors, antibodies to IL-6 receive 27% of patients, antibodies to IL-1 -6,25%. It is worth noting that when using biological agents in 60% of cases, the criterion of an inactive disease was achieved by 4-5 months, which was characterized by the absence of acute inflammatory symptoms, normalization of ESR and CRP. Monitoring of patients with JIA receiving biological agents required the conduct of a number of educational activities for medical personnel, the creation of an additional methodological base. For further training of young specialists at the regional medical university, a program of an additional educational course in pediatric rheumatology was developed and introduced. A regional patient organization was established and also required a set of information activities by the medical community.

Conclusion: In the Saratov region of the Russian Federation, about 20% of patients with JIA receive biological therapy, which corresponds to the average indicators according to the literature. In the structure of the biological drugs used, the group of TNF-alpha inhibitors is preserved -67%. The introduction of modern methods of treatment using biological agents in JIA has significantly increased the effectiveness of treatment, but it required the organization of additional information support for medical personnel.

Disclosure of Interest: None declared Introduction: Immunogenicity and development of anti-drug antibodies have been associated with treatment failure and adverse events during biologic treatment. Anti-drug antibodies (ADAs) have been reported in 21% of Juvenile Idiopathic Arthritis patients treated with Adalimumab. However, their role in reducing adalimumab efficacy is still debated due to conflicting results. No study has been directed toward identification of neutralizing ADAs in paediatric rheumatic disorders. Objectives: Aim of our study was to detect ADAs, along with their clinical relevance, using a new theranostic peptide-base assay in a cohort of children with inflammatory chronic diseases on Adalimumab treatment. Methods: Six candidate Adalimumab derived peptide antigens (HC-CDR1, HC CDR2, HC CDR3, LC CDR1, LC CDR 2, LC CDR3) have been developed and optimized to be tested. Their performance has been compared with commercial ELISA kit and a SPR-based optical assay (Biacore®). Assays have been performed in sera of a cohort of children receiving Adalimumab due to an inflammatory chronic disease. Mean age, disease duration, concomitant treatment with methotrexate (MTX), ANA positivity, disease activity parameters and scores at the time of ADA determination have been recorded. Chisquare, and Fisher exact test were used to compare data. Pearson's and Spearman's correlation tests were used to determine correlation coefficients for entered variables. Results: Eighteen (14 F, median age 12.6, range 3.8-16, yrs) patients were enrolled: 16 affected by Juvenile Idiopathic Arthritis, 7 of whom complicated by JIA -associated chronic uveitis, and 2 patients affected by chronic idiopathic uveitis. Peptide assay revealed ADAs in 8 children, Biacore in 6, commercial Elisa in 5. Of note, we found total concordance among the 3 tests just in 2 patients. No significant correlation has been proven among the 3 ADA determinations. Biacore and ELISA determination showed significant concordance (r s : 0.72, p<0.006). The presence of HC CDR3 and LC CDR 3 resulted significantly correlated with disease activity (r s : 0.57, p<0.05), and, inversely, with disease remission on treatment (r s = -0.523, p<0.05). No patient experienced severe adverse events and no correlation with ADAs has been revealed Conclusion: In chronic rheumatic disorders, novel reliable methods are urgently required to guide clinical decision and support decisions about switching within or between drugs in refractory children. The 3 different methods, since based on different antigenic probes, detect different antibody populations. The present peptide-based assays might contribute to identify neutralizing ADAs in patients treated with Adalimumab. Further validation in larger cohort is required. Introduction: Non-bacterial multifocal osteomyelitis (NBO) is a rare polygenic autoinflammatory disease, which is difficult to diagnose and treat. Because of combination of bone lesions with arthritis and/ or axial skeleton damage in most cases the diagnosis of juvenile idiopathic arthritis (JIA) or juvenile ankylosing spondylitis (JAS) may be establish as a concurrent diagnosis, so this allows to legal use of Biologics (BA) for the treatment. Objectives: To analyze the single center experience of clinical and laboratory features of multifocal NBO in patients (pts) who were treated by BA for the last 8 years.

Methods: The study involved a retrospective cohort of multifocal NBO pts treated by different BA in our clinic from 2013 to 2020. All of them underwent standard rheumatological examination. In order to examine all localizations of the bone damage, a scintigraphy and/ or "whole body" MRI scan was performed.

Results: Among the whole group of pts with NBO (n=40) we identified 13 pts treated by BA (TNF-inhibitors only). The majority were girls (n=9, 69 %). Age at disease onset was 10.2 years in average (Me 10.2 range 1.3-16.5). For legal reason of BA administration, we classified our patients according to rheumatological features as JIA or JAS. 7 pts had JIA (5 girls), 6 pts had JAS (4 girls). Among 13 pts 9 had oligoarthritis (69%), 4 had polyarthritis of low limbs (hip, knee, ankle). Axial involvement was represented by active erosive sacroiliitis with deep bone marrow edema on MRI scan in 9 pts (69%), active spondylitis of several bodies in thoracic spinein 2; erosive arthritis with partial ankyloses of facet joints of neck in 3 pts, multiple syndesmophytes in 1 girl. We found that definite axial lesions in NBO developed in very young children (in 2 y.old at minimum), much earlier than in "idiopathic" JAS. HLA B27 was presented in 5 pts (39%), 5 pts had ANA in high titer (all of those HLA B27-negative). The pts had bone lesions in different parts of skeleton: vertebral bodies -5 pts, clavicle -1, sternum, ribs -1, extremities bones, metaphysic mostly (tibial, fibular -7 pts), sacroiliac region -4 pts. Extraskeletal manifestations were observed in 3 pts, one in each condition -uveitis, psoriasis pustulosus, acnae conglobate. In a girl with very severe course of disease, not responded to any therapy NBO was combined with familial Mediterranean fever. High level of laboratory activity were detected before biologics in 10 pts (77%): ESR acceleration up to 60 mm/h, increase of CRP up to 80 mg/l. Treatment included NSAIDS (all), methotrexate (7 pts), sulfasalazine (6 pts, but it was withdrawn in all pts), bisphosphonates (1 pt), prednisolone (3 pts). Because of high activity of NBO with appearance of new bone lesions and persistent arthritis TNF inhibitors were administrated: etanercept in 10 pts, adalimumab -4 (2 as first line, 2second line), golimumab -1. At the start of BA the average age was 13.7 years (range 7.2-17.9); mean disease duration was 3,4 years (range 0.3-8.1). There were 2 cases of withdrawals. Due to inefficacy etanercept was switched to adalimumab. Disease activity decreasing was reached in the most of the patients (12 from 13). Among them 2 pts developed the whole remission with resolving of active arthritis and bone marrow edema spots. Skin lesions (psoriasis pustulosis and acnae conglobate) were significantly improved. There were no adverse events during the TNF therapy. Conclusion: Our experience of the therapy with TNF inhibitors in patients with high NBO activity has shown that this is a good and safe therapeutic option that is expected to prevent progression and bone destruction. . AE were reported for 71.7% of patients, most within 24 to 48 hours after the first or second injection: flu-like symptoms (57.5%), hypocalcaemia (37.5%) and hypophosphatemia (20%). Underweight patients (body mass index < 18.5 kg/m²) accounted for 50% of hypocalcaemia. The frequency of all the AE not significantly decreased with the reduction of the first dose. Only one serious hyponatremia occurred corresponding to a patient with renal failure before treatment. Conclusion: Our results were similar to those previously published: bisphosphonates are safe for osteoporosis in children. In the literature, SAE are very rare in children, being limited to anecdotal osteopetrosis in cases of higher doses and long-term treatment, and delayed bone healing. Anecdotal osteonecrosis of the jaw in adults has never been described in children. The use of bisphosphonates beforehand requires dietary measures (vitamin D and calcium supplementation). Furthers systematic collection on efficacy and safety parameters for each Bisphosphonates drug should confirm these data. Introduction: The use of biosimilars in rheumatology has increased significantly over the last 5 years and has resulted in considerable cost savings.

Objectives: To assess the effectiveness and tolerability of the Adalimumab biosimilar ABP 501 in patients with JIA. Methods: A database of patients prescribed Adalimumab in our service has been screened to identify patients with JIA, who switched from the originator to the biosimilar. Only patients who had a clinical review since they had started the biosimilar were included. A paired-samples t-test was conducted to compare the number of active joints at the clinic appointment before and after the initiation of the biosimilar treatment. The frequency and type of side effects, the clinical response and the number of patients who switched back to the originator have been collected. Results: Sixty-one patients who switched to the biosimilar ABP 501 between February 2019 and February 2020 were included. They were comprised of 30 enthesitis-related arthritis (ERA), 13 polyarthritis, 9 oligoarthritis, 6 psoriatic and 3 systemic JIA patients. Their baseline characteristics and outcomes are summarised in Table. The mean duration of follow-up after the switch to biosimilar was 10 months (range 2-23). Eleven patients (18%) reported side effects; the most common side effect (n=7, 63.6%) was injection site reactions and the remaining 4 consisted of anaphylaxis, druginduced lupus, dizziness and bone pain, respectively. Seven patients (11.5%) reverted to the Adalimumab originator, 4 as a result of side effects, 3 because of ineffectiveness and one patient for both reasons. In addition, 3 patients were changed to a different biologic, one patient due to allergy to both the originator and biosimilar and the other two patients had active disease on the originator and biosimilar Adalimumab. Two patients stopped the biosimilar and remained off any biologic, in the first case this was due to a side effect and in the second case it was patient's choice. On the whole, 78.7% of patients had remained on ABP 501 at their last visit. There was no significant difference in the active joint count before the biosimilar was started (mean 0.55+/-1.11) and after the switch (mean 0.6+/-1.59), (p= 0.855). Introduction: Golimumab (GOL) is approved for polyarticular juvenile idiopathic arthritis (pJIA) in patients of ≥2years but long-term safety data are limited.

Objectives: Prospective monitoring of long-term safety and effectiveness of GOL in routine care using the BIKER-registry. Methods: Baseline demographics, clinical characteristics, disease activity and safety parameters were compared to a contemporary 1:2 matched control cohort using alternative TNF inhibitors or methotrexate without exposure to a biologic. Efficacy outcomes were JADAS10, joint counts and functional status. Safety assessments were based on adverse events (AE) reports.

Results: In this ongoing study, 65 pts initiating GOL were matched to 130 with alternative TNFi and 65 biologic-naïve pts. Pts starting GOL had a longer disease duration (p<0.0001) and use of GOL was significantly more often second line (84.6% vs 22.3%, p< 0.0001) and thus disease activity was lower at baseline. Pts in the GOL cohort used less corticosteroids, otherwise patients were comparable with pts treated with other TNFi (Table 1 ).

In GOL treated ps a marked clinical response was noted at 6 months and beyond, indicating the effectiveness of GOL in the treatment of pJIA. A significant decrease of the mean JADAS 10 11.3 to 5.3 (p= 0.0008) after 6 months of treatment was observed, as well as JIA ACR 30/50/70/90 response rates of 61/59/42/29%. JADAS remission and minimal disease activity was observed in 27% and 53.7% after 6 months and in 39% and 54% after 12 months of treatment.

Rates of AE, SAE and infectious AE were comparable in the GOL cohort (87.5/100PY, 3.4/100PY and 11.1/100PY), the alternative TNFi cohort (92.3/100PY, 2.9/100PY and 9.7/100PY) and the MTX only cohort (121.2/100PY, 2.1/100PY and 18.5/100PY). SAE reported in the GOL cohort were flares of uveitis and of JIA (each 1) and fibromyalgia syndrome (1) . SAE reported in the alternative TNF cohort was two serious infections (both influenza), one knee ligament injury, one flare of arthritis and one hyperventilation . No case of pregnancy, malignancy or death was reported.

Conclusion: Golimumab seems an effective in treatment of pJIA. Tolerability was acceptable and comparable to alternative TNFi or MTX. Recruitment to the project is ongoing.

Disclosure of Interest None declared Introduction: Methotrexate (MTX) is one of the most commonly used disease-modifying anti-rheumatic drug in rheumatology practice. It has some side effects that can impair quality of life. The most common of them is associated with the gastrointestinal tract.

Objectives: The aim of the study is to evaluate and compare the frequency of methotrexate intolerance in adult and pediatric patients.

Methods: Patients with rheumatologic diseases followed in Hacettepe University Pediatric Rheumatology and Rheumatology departments who used oral or parenteral methotrexate for at least 3 months were included in the study. Methotrexate intolerance was assessed using 'Methotrexate Intolerance Severity Score (MISS) questionnaire. The MISS questionnaire consisted of 5 parts: abdominal pain, nausea, vomiting, fatigue and behavioral symptoms. The patients scored the severity of each symptom separately; 0: no symptoms, 1: mild symptoms, 2: moderate symptoms, 3: severe symptoms. A total score of 6 or more was defined as MTX intolerance. Visual analogue scale (VAS) ranging from 0 cm to 10 cm was performed to each patient concurrently with the MISS questionnaire. In the pediatric patient group, MISS questionnaire and VAS assessment were applied to both patients and families.

Results: A total of 100 patients, 50 of whom were children, enrolled in the study. The mean age for children and adults were 11.78 (± 3.4) and 52.9 (± 11.8) respectively. The most frequent diagnosis of patients was juvenile idiopathic arthritis (78.0%) in children and rheumatoid arthritis in adults (68.0%). The mean MTX dose in adults and pediatric group was 12.5 (±3) mg vs 14.5 (± 3.6) mg (p: 0.004). The prevalence of MTX intolerance in children and adults were 66.0% (n:33) and 14.0% (n:7) respectively. The mean MISS score in the pediatric group was higher compared with the adults (12.4±9.4 vs 1.84±4.5, p<0.001). Similarly, the mean VAS scores were higher in pediatric group (1.2±2.4 vs 4.2±3.2 (p<0.001)). There was a strong correlation between MISS and VAS scores between family and child evaluations (p <0.01, r = 0.95 / p <0.01, r = 0.94). Abdominal pain, nausea, vomiting and behavioral symptoms were observed more frequently in children compared to adults. Results: 3(4%) out of 73 patients were diagnosed with psoriasis denovo. One patient was treated with ADA (a girl with undifferentiated arthritis who had positive HLA-B 27, ANF and family history of psoriasis -her grandmother had psoriasis), 2 patients were treated with ETA (both female, one patient had undifferentiated arthritis, the other had enthesitis-related arthritis; both patients had positive HLA -B 27 and ANF negative). 2 patients achieved significant improvement after changing TNFalpha inhibitor (1-ADA, 1-ETA), 1 patient (was treated with ETA) had significant improvement after discontinuation of biological therapy.

Conclusion: This single-center observational study demonstrates the possibility of developing psoriasis de-novo in patients with JIA receiving TNF-alpha inhibitors. Although more extensive research is needed, our data suggest that discontinuing the TNF-alpha inhibitor or switching to another TNFalpha inhibitor in patients with psoriasis de-novo should be considered as a treatment strategy in such cases. Objectives: Long-term surveillance of patients newly initiating TOC treatment for at least 5 years compared to a cohort of patients newly initiating a comparator biologic using the BIKER-registry. Methods: Baseline demographics, clinical characteristics and disease activity, efficacy and safety parameters were compared. Efficacy outcomes were JADAS10, joint counts and functional status Safety was assessed by adverse events (AE) reports. Results: 161 patients with 161 matched controls have been recruited. Patients starting on TOC were older at treatment start (12.1 vs. 10.1 years (y); p<0.0001) and had a longer disease duration (p< 0.0001). TOC was significantly more often a second line biologic (p< 0.0001). Baseline JADAS10 (17+/-10 vs 15+/-6), CHAQ-DI (0.63+/-0.63 vs 0.65+/-0,64), ESR 18+/-15 mm/h vs. 21+/-21 mm/h and active joint counts (7+/-7 vs. 6+/-5) were comparable. Upon TOC a substantial response with a significant reduction in JADAS 10 from 16.8 to 3.4 (p<0.0001) after 12 months of treatment was observed. There were no significant differences between patients from the TOC cohort and their matched controls in the JIA ACR 30/50/70/90 criteria, JADAS 10, JADAS remission and minimal disease activity was reached by comparable numbers (TOC 37% and 58%; control cohort 37% and 60%). The total number of AE was comparable (TOC cohort n=201 AE; (77/ 100PY); control cohort n=207; (65/100PY; RR 1.2; 95%CI 0.99-1.4). More serious AE (SAE) were reported with TOC. Serious infections were documented at lower frequency with TOC. Uveitis events were documented at significantly higher frequency with TNF inhibitors most likely due to a selection bias (Table 1) . SAE with TOC were depression (n=3) in 2 with suicidal intent, exacerbation of JIA (n=2), septic arthritis, gastrointestinal infection, abdominal pain, colitis, paronychia and fracture. SAE in the control cohort were depression, osteomyelitis, gastrointestinal infection and disease flare. No significant differences regarding cytopenias and elevated transaminases were observed. No gastrointestinal perforation, no vascular events and no deaths occurred. Conclusion: TOC was effective and comparable to treatment with alternative biologics. Tolerability was acceptable. As TOC was given as a second-line biologic in the vast majority of patients comparisons between the 2 cohorts have to be interpreted carefully. Observation is ongoing. Conclusion: In this retrospective cohort study in pediatric patients on RTX-treatment, we found undetectable low drug levels in ADApositive patients, indicative for their neutralizing capacity. Consequently, the lack of B-cel depletion leads to reduced treatment efficacy. Patients with SLE seem more susceptible to develop ADA. If ADA are detected, continuation of treatment seems non-effective and changing medication is advised. Certainly when considering that, in this study, anaphylactic reactions only occurred in ADA-positive patients.

None declared Objectives: The aim of this study was to evaluate retrospectively the long-term efficacy and safety of adalimumab in patients with JIAassociated uveitis. Methods: We have retrospectively analysed nineteen JIA patiens data with associated uveitis from our centre registry between 2010 and 2020, treated with adalimumab after failure of treatment with corticosteroids and metotrexate. Demografic data and blood samples were collected at different time points while uveitis activity was evaluated by slit-lamp biomicroscopy. Adverse events were recorded. Results: Registry records provided 10 years follow up of 19 JIA patients data with associated uveitis. Eleven patients were females (57.90 %) diagnosed as oligo/extended oligoarticular JIA while eight (42.10 %) were males diagnosed as enthesitis related arthritis (ERA). Before adalimumab was prescribed, all patients were previously treated with metotrexate during 3.5 years in avarage dose of 10 mg/ m 2 weekly. The mean uveitis duration, before adalimumab administration was 9 months. Ten years long follow up period have showed that there were no new relapsis of uveitis while patients were receiving adalimumab and metotrexate. All of our patients were able to gradually tapper and stop treatment with topical steroids two months after adalimumab commencing. Seven patients were able to stop biological treatment after 4.3 years of adalimumab usage. Uveitis relapsed three monts after the adalimumab discontinuation only in one patient. Two patient were lost to follow up during the transitional period. No serious adverse events were recorded. Conclusion: During the long term follow up period adalimumab have shown good efficacy and safety profile in JIA patients with active inflammatory ocular disease. Introduction: Post-streptococcal syndrome is a systemic immunemediated complication of beta-haemolytic streptococci infection, mostly seen as post-streptococcal arthritis, rheumatic fever or glomerulonephritis. Uveitis is an uncommon manifestation of this syndrome.

Objectives: Case report Methods: Case report Results: A previously healthy 7-year-old female was admitted at the emergency department with prolonged fever, arthritis and red eye. She had a 4-month history of febrile episodes every two weeks, with axillary temperature ranging from 37,8 to 39ºC. Migratory arthralgia affecting both knees and tibiotarsal joints showed up two months after the fever onset and worsened in the previous week, with refusal to walk. Non-painful bilateral red eye for several weeks was mentioned. Other symptoms were absent. Recent infections were denied and family history was irrelevant. Physical examination revealed lower limb muscular atrophy, knees pain and impaired function and bilateral tibiotarsal arthritis with inability to walk. Ophthalmological observation showed a bilateral non-granulomatous anterior uveitis. Sequential laboratory work up revealed a maximum eritrocitary sedimentation rate of 135 mm/h, maximum c-reactive protein of 5,3 mg/dL, microcytic hypochromic anemia, positive antistreptolysin O titer (ASOT) (initial result of 1250 that increased to 2500 in 4 weeks and later decreased to 500) and negative anti-nuclear antibodies. Cardiac involvement was excluded. The diagnosis of rheumatic fever with concomitant poststreptococcal uveitis was assumed and the patient was treated with oral and topical ophthalmic corticosteroids with prompt clinical resolution of fever, acute polyarthritis and uveitis. No relapse occurred in a 5-year follow-up. Conclusion: Juvenile idiopathic arthritis (JIA) is the most common cause of uveitis in childhood. Although our patient clinical course could initially raise the possibility of systemic JIA (sJIA), the criteria that define this entity weren't all present and clinical and laboratory findings were more supportive of rheumatic fever. Besides, uveitis occurs exceptionally in sJIA, which turned this diagnosis even less reasonable. In our Rheumatology Unit, among 563 patients diagnosed with JIA in 32 years, 89 had uveitis. However, in the group of 51 patients with sJIA only one had ocular involvement, a boy with isolated vitritis. Post-streptococcal uveitis (PSU) typically presents as bilateral, non-granulomatous anterior uveitis, as described in this case. As streptococcal infection is very common among children and many patients may experience subclinical infection. PSU should be considered in all patients with uveitis along with positive ASOT and negative routine investigations for other causes. Although PSU has been described in literature, to the best of our knowledge, this is the first reported case of concomitant rheumatic fever and PSU. . ADA was first tapered to every 3 weeks by 76% of the responders and then to every 4 weeks by 49% before discontinuing. Fewer respondents used or tapered IFX, TOC or ABA. Around 65% tapered the interval and 20% tapered the dose and interval for ABA, 26% for TOC and 37% IFX There were differences in the duration of tapering prior to discontinuation of specific medications. For ADA it was 6 months in 62%, 12 months in 36% ,and 24 months in 10%. For IFX it was 6 months in 27%, 12 months in 45%, and 24 months in 33%. For TOC it was 40% after 4 weeks, 87% after 6 weeks and 53% after 24 weeks. For ABA i.v. it was 30% after 8 weeks, and 90% after 12 weeks. If combination therapy was used, 36% tapered the bDMARD first, 62% csDMARD first, and 12% both simultaneously.

Conclusion: This is the first survey to describe "real world" medication tapering and discontinuation practices of pediatric rheumatologists and ophthalmologists globally. Most physicians start to taper medication after 24 months of remission on medication and discontinue after the 6 to 12 months of tapering.

We would like to thank all the participating colleagues, who took time to fill out our surve Introduction: JIA-associated uveitis (JIA-U) occurs in 10-20% of children with Juvenile Idiopathic Arthritis (JIA) and typically asymptomatic, and sight-threatening complications occur in 50% of children, (i.e. cataracts, vision loss). Frequent ophthalmic examinations are important for early diagnosis and monitoring of uveitis activity. Even after uveitis is controlled, risk of disease exacerbation still exists. Therefore, frequent ophthalmic screening and monitoring is important for detection and management of JIA-associated uveitis (JIA-U). S100 proteins, cytokines, and chemokines detected in aqueous humor of patients with uveitis are also detected in tears. Biomarker discovery using tears is promising since collection is noninvasive, feasible, well-tolerated, and close to the target organ. Objectives: We aim to determine if S100 proteins, cytokines, and chemokines levels differ in tears of children with JIA and JIA-U and in children with JIA-U by uveitis activity. Methods: Tears were collected using Schirmer strips from children ≥5 years old with oligo-or polyarticular RF negative JIA with (JIA-U) and without uveitis (JIA-no-U), and in children with JIA-U at time of active and inactive eye disease. Activity was defined by Standardization of Uveitis Nomenclature (SUN) criteria. Active uveitis was anterior chamber inflammation grade ≥0.5+ cells. S100A8, A9, and A12 were measured by ELISA, and IL-18, IL-8, IP-10, MCP-1, RANT ES, and sICAM-1 by Luminex assays. Biomarker levels were compared in children with 1) JIA-no-U (n=8) to active JIA-U (n=8), and 2) JIA-U (n=8) at time of active and inactive uveitis.

Results: Children with JIA-no-U and JIA-U were matched by JIA subtype and arthritis activity. They had primarily oligoarticular JIA (63%), active arthritis (25%), and were on systemic medication (75%). At time of active uveitis, 75% had grade 0.5+, and 25% had 1+ and mean interval between time of active and inactive disease was 11 months. We found that levels of biomarkers in tears of children with JIA-no-U compared to active JIA-U were similar. Although not statistically significant, levels of S100A12 (mean difference 12,190 pg/ML [95% CI -4847 to 29,227], P= 0.14) and sICAM-1 (5329 pg/ML [95% CI -5372 to 16,031], P=0.28) were higher when uveitis was active compared to inactive. Conclusion: Our results suggest that S100A12 and sICAM-1 are potential biomarkers of uveitis activity in JIA-U, but not uveitis diagnosis. Thus, neutrophils may play a role in the pathogenesis of anterior uveitis which has been reported in an animal model of acute anterior uveitis. Identifying biomarkers using tears provides a noninvasive and objective method of monitoring uveitis. Limitations are our heterogeneous cohort that varied by arthritis severity and immunosuppression, and minimally active uveitis. We were underpowered to detect statistically significant differences and continue to collect tears prospectively in children with JIA-U with goal of n=28. Despite low uveitis activity, we were still able to detect differences. Further studies in larger and diverse cohorts are necessary to assess the role of S100A12 and sICAM-1 in JIA-U. Objectives: To report an extremely rare presentation of GPA in a 12 year old with acute digital ischemia.

A 12 year old boy, with a background of poorly controlled type 1 diabetes and hypothyroidism, initially presented to hospital unwell with diabetic ketoacidosis. Treatment was initiated promptly with good response. Furthermore, he was found to have weight loss, productive cough and hearing loss over the past 3 months. He was haemodynamically stable, but very pale and cachectic. He had reduced air entry and crackles on the right. There was hypertonia and clonus in his lower limbs. Blood tests showed microcytic hypochromic anaemia (Hb 82g/L), normal white cell count, thrombocytosis and raised inflammatory markers (CRP 138mg/L and ESR 68 mm/hr). His chest x-ray showed enlargement of the right hilum with consolidation/ atelectasis extending into the middle and lower lobes. MRI scans of head and spine were normal apart from fluid opacification in the paranasal sinuses. He was screened for infections including Tuberculosis and started on intravenous antibiotics. On day 13, he developed painful bluish discolouration of his left hand, particularly his thumb, index and middle fingers. His left radial and brachial pulses weren't palpable. A heparin infusion was started.

A Doppler scan showed occlusion of radial and ulnar arteries proximal to the wrist with no clear thrombus.

He had a CT thoracic aorta with contrast which showed proximal left radial artery occlusion and distal ulnar artery occlusion with no evidence of proximal embolic source or vasculitis. It showed multiple perihilar masses (lymph nodes) in the right lung and peripheral parenchymal masses in both lungs, suggestive of atypical infection or connective tissue disease. Blood tests still showed raised inflammatory markers(CRP 107mg/L, ESR 86 mm/hr and platelets 658 10 9 /L). An autoantibody screen showed positive ANCA with strongly positive anti PR3(>100 U/mL); other autoantibodies, including ANA, ds DNA and anti-phospholipid antibodies, were negative. He developed further ischaemia with bluish, painful discoloration of his right foot, especially right great toe, with a weakly palpable dorsalis pedis pulse. Doppler scan revealed occlusion/narrowing of the posterior tibial artery 6cm proximal to the ankle. Following vascular team advice, he was started on ilioprost infusion to aid reperfusion of the extremities involved, with good results. Based on clinical and lab features of systemic inflammation, evidence of upper airway involvement(bilateral conductive hearing loss and sinusitis on MRI scan), parenchymal lesions on CT chest and strong PR3 positivity, a diagnosis of GPA was made.

Results: Our patient responded well to therapy including multiple pulses of high dose methylprednisolone and cyclophosphamide, with improvement of all organs involved and no further digital ischemia.

Conclusion: Although GPA is very rare in children, it is associated with high morbidity and mortality. Many studies show that the spectrum of paediatric GPA is not vastly different from adults, except for higher gender bias towards female, more constitutional and musculoskeletal symptoms and higher risk of subglottic stenosis.

Although there are a handful of case reports of digital ischaemia in adults with GPA, to our knowledge this is the first case report of acute digital ischaemia in paediatric GPA. Early diagnosis and prompt treatment with a multidisciplinary team approach is paramount for good outcome. Introduction: Adenosine deaminase-2 deficiency (DADA2) is a monogenic vasculitis syndrome whose presentation ranges from recurrent fevers and livedo reticularis to systemic vasculitis, hematologic and immunologic abnormalities, and early-onset stroke. It is characterized by biallelic loss-of-function mutations in the encoding gene of ADA2 protein and low levels of ADA2 enzymatic activity in the peripheral blood. The genotype and phenotype features of DADA2 has a wide spectrum. Treatment with anti-TNF inhibitors is effective in controlling vascular inflammation and reducing strokes.

Objectives: To describe two sisters with different presentations of DADA2 and a deletion mutation on exon 7 of the ADA2 gene. Methods: Medical data was used to describe the clinical manifestations of two siblings. Parental informed consent was obtained.

Results: Patient 1: A 10-year-old female had presented with fever, rash, arthralgia, hepatosplenomegaly, and coombs positive autoimmune hemolytic anemia (AIHA) at the age of 7 years. She had been followed with a suspected diagnosis of systemic lupus erythematosus (SLE) and steroids, azathioprine, mycophenolate mofetil had been used. Her ANA and complement levels were normal. Because of unmet classification criteria of SLE, genetic testing had been done, and no mutation found in the ADA2 gene. Cranial MR and MR angiography was normal. She was referred to our clinic after 2.5 years of the first manifestation. Physical examination revealed Raynaud phenomenon on both hands and feet, livedo reticularis, arthritis, and splenomegaly. Laboratory tests indicated an increase in acute phase reactants, CD19, CD20, and switched memory B cell lymphopenia, and hypogammaglobulinemia. Because of prolonged fevers, a thorax CT was obtained and aneurisms of the renal artery were seen. Abdominal CT angiography indicated multiple aneurysms of both renal, intercostal, and hepatic arteries. Repeated genetic analysis of the ADA2 gene showed a homozygous deletion mutation on exon 7. She has been followed on anti-TNF and IV immunoglobulin without severe symptoms for a year. Patient 2: The older sister had been followed with a diagnosis of familial Mediterranean fever with E148Q heterozygous mutation because of recurrent fever, abdominal pain, erysipelas-like erythema, elevated acute phase reactants, and splenomegaly. She did not have any other cutaneous or systemic findings. Because of parental consanguinity, the ADA2 gene was analyzed and a homozygous deletion mutation on exon 7 was found. She has been followed without any symptoms after anti-TNF treatment. Throat swab was negative. Abdomen ultrasound showed bowel wall thickening, testis ultrasound was normal. HSP diagnosis was confirmed. Methylprednisolone iv was administered for three days, then oral prednisone was started. Purpuric lesions, abdominal pain persisted, so we decided to add MMF (600mg/ m 2 /day) and prednisone was tapered in a month.

Results: Thanks to MMF vasculitis lesions and abdominal symptoms disappeared in few days. MMF was continued for a month, tapered in 6 months. There was no evidence of relapse in a 6 months follow up. Conclusion: These cases suggest that MMF may be useful to induce and maintain remission of recurrent HSP with gastrointestinal involvement. Multicenter clinical trials with long-term follow up to confirm the efficacy of MMF in the treatment of HSP with gastrointestinal involvement are needed. Introduction: Henoch-Schönlein purpura (HSP), the most common childhood vasculitis. Cholecystitis is extremely rare in patients with HSP. This is the first case of a Libyan child presenting with HSP complicated by calculus cholecystitis HSP nephritis. Objectives: our aim is to present an unusual case of gall bladder involvement in an 8-year-old Libyan female affected by HSP. Methods: a case reports study Results: : we report an unusual case of gall bladder involvement in an 8-year-old Libyan female with HSP. She was referred to a rheumatology clinic due to HSP with chronic calculus cholecystitis and distended small bowel with fluid-like fecal material with no evidence of intussusception on an abdominal ultrasound. The patient had a one-month history of abdominal pain, purpuric lesion on lower limbs and swelling in both feet. She was admitted 3 times to another hospital before being referred to the rheumatology clinic. An abdominal sonography revealed a distended small bowel with fluid-like fecal material with no evidence of intussusception and chronic calculus cholecystitis; they treated her with Urosdoxycholic acid tab at 250mg per day and ibuprofen syrup. Then referred to our rheumatology clinic. After 40 days, she showed a purpuric rash over her lower extremities, mainly over her thighs and buttocks, microscopic hematuria, no arthritis, no fever, no abdominal pain; her blood pressure was normal at90\55mmHg, and she had normal laboratory tests (CBC, WBC 7.7, HGB 10.8, Platelets 356 ESR 20ml\hour, CRP 1mg\dl was negative, C3 was 150mg\dl within normal range 90-180mg\dl, C4 was 35.4 mg\dl within normal range 10-40, ANCA, ANA, as well AntidsDNA Ab yielded negative, Antistreptolysin-O (ASO) titer was 250 Todd , LFT included total bilirubin , direct , indirect GPT,GOT, U, creatinine ) except urine routine showed mild microscopic hematuria RBC 100 HPF , protein was Nil ) urine for pro-tein

Disclosure of Interest None declared P161 Correlation of serum neopterin levels with disease activity and

Moneta 1 1 Division of Rheumatology, IRCCS, Ospedale Pediatrico Bambino Gesù, Roma; 2 University of Genova

A multinational study of thrombotic microangiopathy in macrophage syndrome

Clinical impact of a targeted next-generation sequencing gene panel for autoinflammation and vasculitis

Laboratory biomarkers to facilitate differential diagnosis between measles and kawasaki disease in a pediatric emergency room: A retrospective study

A rare case of measles-associated hemophagocytic lymphohistiocytosis in an infant. Cureus

Children's interstitial and diffuse lung disease

Respiratory Complications of the rheumatological diseases in childhood

on behalf of Dr Nishant Dharsandiya and Dr J.P. Keshrani Paediatric Rheumatology & Immunology, Dev Children's Hospital

Arthritis Care Res (Hoboken)

Disclosure of Interest: None declared

Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis

Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome

double-blind, placebo-controlled study of anakinra in pediatric and adult patients with Still's disease L. Schanberg 1 , P. Nigrovic 2

Duke Children's Hospital & Health Center, Durham; 2 Boston Children's Hospital, Boston; 3 Children's Mercy Kansas City, Kansas City; 4 University of Alabama at Birmingham, Birmingham; 5 Nationwide Children's Hospital, Columbus; 6 University of Iowa Hospitals and Clinics

Children and adolescents with SLE: not just little adults

Severe disease presentation and poor outcomes among pediatric systemic lupus erythematosus patients in South Africa

The Checklist Manifesto: How to Get Things Right

Clinical review: Checklists -translating evidence into practice

The WHO surgical safety checklist: a review

Pehlivanoğlu 3 P283 Gastrointestinal Henoch-Schönlein purpura treated with mycophenolate mofetil: description of two case reports

Venous vessel wall thickness in lower extremity is increased in male patients with Behcet's disease

Increased vein wall thickness in Behçet disease

Polyarteritis nodosa: A contemporary overview

EULAR / PRINTO / PRES criteria for Henoch -Schönlein purpura , childhood polyarteritis nodosa , childhood Wegener granulomatosis and childhood Takayasu arteritis : Ankara

Six patients were treated with canakinumab and 2 patients with anakinra. Conclusion: It is known that excessive production of IL-1β can cause inflammatory bone loss and abnormality. Vitamin D deficiency and osteopenia/ osteoporosis may cause additional musculoskeletal problems besides arthritis and joint destruction in CAPS. We think that Ca metabolism and bone mineral density measurements should be a part of routine controls in patients with CAPS. Disclosure of Interest None declared AB007 Clinical and genetic features of patients with periodic syndrome associated with mutation of the tumor necrosis factor receptor gene and juvenile arthritis having mutations in TNFRSF1A gene M

Active arthritis in 8/9, it was poly in 2, oligo in 6. When assessing the clinical symptoms and laboratory activity of patients with JIA, it was revealed that in the onset of the disease, systemic manifestations were observed in 8/12: fever in 8/12, rash in 4/12, hepatosplenomegaly in 5/ 12, pneumonitis in 2/12, carditis in 1/12 and lymphadenopathy in 5/12. High laboratory activity was recorded in 11/12. Active arthritis in 10/12, it was polys in 4, oligo in 6. In all 100% of patients, the nucleotide variants of the TNFRSF1A gene were identified in the study. 9/21 of patients were diagnosed with TRAPS. The most frequent heterozygous variant of TNFRSF1A gene with nucleotide substitution of c.362G>A was found in 7/9 of patients, in 1/9 of patients it was found homozygous variant with nucleotide substitution of c.362G>A, in 1/9 of children it was found heterozygous variant with deletion of c.337_339del. All of these variants are pathogenic. 12/21 of patients were diagnosed with JA: juvenile arthritis with a systemic onset was in 7/12, paucarticular arthritis was in 2/12, in 1/12 it was poly RF-and in 1/12 it was psoriatic arthritis. It is worth noting to note that in 6/12 a heterozygous version of the TNFRSF1A gene was detected with a nucleotide substitution c.362G> A, however, considering the absence of clinical manifestations of autoinflammatory disease and active articular syndrome in these patients, children were diagnosed with JA. In addition

Dvoryakovskaya: None declared, A. Mamutova Speaker Bureau of: Novartis, K. Isayeva: None declared, R. Denisova Speaker Bureau of: Novartis

COVID-19 and relapsing Kawasaki disease: a case report during the pandemia M. C. Maggio

AB009 Introduction: The pandemia of COVID-19 remains a global health alarm with high incidence of lethality, especially in older age groups who suffer from underlying medical conditions. However, children are less likely to manifest severe conditions. Objectives: COVID-19 was correlated to a higher incidence and a suspected increased risk of Kawasaki Disease (KD) in children

Anamnestic records revealed a previous KD, without coronary artery lesions (CAL), 1 year before. Results: He was treated with antibiotics, intravenous infusion of Immunoglobulins (IVIG) (2 gr/Kg), acetylsalicylic acid (ASA) (50 mg/Kg in 4 doses/day) and reached defervescence into 2 days. Echocardiography excluded CAL. The nasopharyngeal swab for SARS-COV-2 was doubt. The second throat swab done the day after IVIG infusion, was negative; however, the third nasopharyngeal swab for SARS-COV-2, done 4 days after IVIG infusion, was positive. Chest x-ray showed a significant lung interstitial thickening. IL-6 levels were < 6.25 pg/ml (n.v. < 6.25 pg/ml). He continued treatment with antibiotics, ASA (5 mg/Kg/day), with the progressive resolution of the clinical symptoms and of the normalization of laboratory findings. Conclusion: The peculiar outcome of the patient is the correlation of COVID-19 with KD, recently reported as associated. KD is considered as a multifactorial autoinflammatory disease, induced by a cytokine hypersecretion with a systemic vasculitis. COVID-19 is considered a cytokine storm syndrome, with a severe systemic vasculitis. SARS-COV-2 infection could be the trigger that could lead to hyperinflammation of KD. The IVIG infusion could explain the transient negative swab for SARS-COV-2, with the successive positive relieve lasting 7 days, and the normal levels of IL-6, detected after IVIG infusion. Relapsing KD is rare (1.7-3.5%); in our patient this event could be triggered by the documented SARS-COV-2 infection. Disclosure of Interest None declared Disclosure of Interest None declared AB012 Spectrum of systemic inflammatory syndrome in children during COVID 19 pandemic in India D. B. Pandya, on behalf of Dr Haresh Dobariya

Pediatric Rheumatology & Immunology, Dev Children's Hospital

AB025 Rituximab for treatment of resistant Paediatric MCTD V. Paisal, S. Compeyrot-Lacassagne Paediatric Rheumatology

The diagnosis and classification of of mixed connective tissue disease

Mixed connective tissue disease in children -case series

The value of Rituximab treatment in primary Sjögren's syndrome

Juvenile Idiopathic Arthritis a multifaceted approach is essential for robust rehabilitation M

Methods: In a retrospective study 92 children (89% girls) aged median (IQR) 4,2 (1,6 -7,6) years with oligoarticular onset JIA without extra-articular manifestations (oligo-JA) who did not received DMARDs were monitored. All children were met ILAR criteria. Ttriamcinolone acetonide (TA) was administered intra-articular at a dose of 20-40 mg with an injection interval of 3-6-12 months which was depended on the activity of the disease. The maximum allowable number of consecutive isolated intra-articular injections (is-IAI) was 3-4. A total of 218 active joints were injected with TA: knees -156 injections, ankles -62 injections. All children were divided into two groups: active / inactive arthritis based on the effectiveness of local corticosteroid treatment. The average follow-up was 48

and physicians' assessment of JIA disease activity

Efficacy is-IAI of TA was no associated significantly with number of active joint of onset oligo-JA, cJADAS10, serum level of CRP mg/ml, ESR mm/h, IL6 pg/ml and TNF-α pg/ml, titer of ANF. The mean inflamed synovial fluid of IL6 levels 2208

Abstracts from conferences and relevant studies were added. RCTs were included if (i) patients were aged ≤ 20 years, (ii) patients had a previous defined pediatric rheumatic diagnosis and (iii) RCT met predefined outcomes. Studies were excluded in case of (i) observational or single arm study or (ii) sample size ≤ 5 patients. Study design, location, duration, treatment, population, sample size, age criteria, gender, concomitant treatments and primary outcome was extracted. Results: Out of 550 screened references, 62 references reporting 35 unique RCTs in PiRD. All 35 RCTs reported efficacy while 34/35 RCTs provided safety outcomes and 15/35 RCTs provided PK data. Ten of 17 reviewed bDMARDs are approved for PiRDs by the Food and Drug Administration (FDA). Of these, seven had ≤ 2 RCTs. The most common intervention was TNF inhibitors (63%)

Treatment with intravenous immunoglobulin (IVIG) significantly reduces the risk of CAAs. However, up to 20% of cases are IVIG resistant with a higher risk of cardiovascular complications. Currently several second-line treatments are available for refractory KD. Nonetheless, the existing literature is still unable to identify which treatment is the most effective. Recent studies suggest that a IL-1 receptor antagonist (anakinra) may be an effective therapy in refractory KD. Objectives: We report the case of a 3 year-old boy diagnosed with KD refractory to conventional treatment, who developed giant CAAs successfully treated with subcutaneous (sc) anakinra. Methods: Case report. Results: A 3 year-old boy was referred to our Pediatric Rheumatology Unit 18 days after the onset of a Typical refractory KD. He had been previously treated at a local hospital with two doses of IVIG (2 g/kg), infused respectively 8 and 11 days after the onset of the fever. Afterwards, given the persisting fever, doses of pulse intravenous (IV) methylprednisolone (MPDN 30 mg/kg/day) have been used for 3 days followed by oral prednisone (2 mg/kg/day). Treatment with Acetylsalicylic Acid (60 mg/Kg/day q8h) was also started. Following a transient defervescence the day after the first IV pulse MPDN, fever relapsed and the echocardiography showed CAAs of Left Main Coronary Artery (LMCA), Left Anterior Descending (LAD) and Right Coronary Artery (RCA)

Rationale and study design for a phase I/IIa trial of anakinra in children with Kawasaki disease and early coronary artery abnormalities (The ANAKID Trial)

The use of interleukin 1 receptor antagonist (anakinra) in Kawasaki disease: A retrospective cases series

There are few reports of acute kidney injury (AKI) in KD, defined as serum creatinine level elevation to more than 1.5 times of baseline level. Objectives: To describe the case of Kawasaki Disease complicated by AKI Methods: A 5-year-old female was admitted to our Rheumatology Unit with persistent fever (6 days), widespread polymorphous exanthema, change in lips and in oral mucosa. Family history was unremarkable. She had no chronic underlying disease nor history of previous hospitalization. At admission, she appeared stable. Body temperature was 38.9°C, O2 saturation was 96% in ambient area, blood pressure was 118/75 mm Hg, heart rate was 90 bpm, respiratory rate was 21 breaths per minute. On examination she presented widespread polymorphous exanthema, changes in lips and in oral mucosa, cervical lymphadenopathy and bilateral conjunctival injection. Results: Exams revealed: white blood cells 11980/μl, Hb 10.4 g/dL, platelets 389.000/μl, albumin 2.5 g/dL, serum sodium 126 mEq/L, serum chloride 90 mEq/L. Transaminases were in normal range. creatinine was 1.5 mg/dL

Disclosure of Interest None declared AB041 Paediatric extra-pulmonary large vessel arteritis, a forme fruste of pediatric Behcet's disease?

We presented two siblings from a consanguineous marriage with different clinical presentations of DADA2. Further, we emphasize that genetic testing should be repeated in the presence of clinical suspicion.

Introduction: There are several scoring systems developed in Japan that are clinically used to stratify high risk KD patients and thus identify the ones that may benefit from early adjunctive therapy. There are increasing reports from all over the world on poor performance of these scores in other ethnic populations. Objectives: The aim of our study was to evaluate the Kobayashi, Egami, Sano and Kawamura scores in our population which is homogenous Caucasian. Methods: Hospital database was retrospectively searched for code M30.3 of the International Classification of Diseases, 10th Revision, Clinical Modification Code: Mucocutaneous lymph node syndrome [Kawasaki] , over the period from January 2006 to December 2019. All patients who were seen in this period for the first time for complete or incomplete Kawasaki disease, as defined by the American Heart Association, were included. We applied IVIG resistance prediction scores (Kobayashi, Sano, Egami and Kawamura scores) to our cohort. Only patients who received 2g/ kg IVIG within the first 10 days of the disease were included in this analysis. The scores of prediction models were calculated for each patient and patients were assigned to high-or low-risk group accordingly. Results: During the study period a total of 169 children were diagnosed with KD (61.5 % males, median age 3.28 years). All of them were Caucasian except one child who was biracial (Caucasian and African American). Among them, 158 children were hospitalized in the acute phase of the disease and 11 children were seen in the subacute phase of the disease. 151 children were followed-up for at least one year to evaluate persistent coronary artery aneurysms (CAA), which were observed in 8 (5.3 %) patients. Among them, 2 were not treated with IVIG and 2 received IVIG after 10 days of illness. 125 patients were treated with IVIG within first 10 days of illness and were included in the calculation of IVIG resistance prediction scores. 24 (19.2 %) were IVIG resistant. Sensitivity of Kobayashi, Sano, Egami and Kawamura scores were 0.53, 0.47, 0.61and 0.58, respectively. Specificity of those scores were 0.77, 0.87, 0.75 and0.58, respectively. We found no difference in demographic or clinical characteristics between IVIG resistant and IVIG responsive patients. Patients with IVIG resistance had significantly higher ALT (p = 0.025), neutrophil-to-lymphocyte ratio (p = 0.036) and lower serum sodium (p = 0.009). Conclusion: By applying the Japanese scores to our population, we were able to identify most of the low-risk, but missed many of the high-risk patients. Our results are consistent with Caucasi n based population studies available to date. Introduction: Varicella zoster virus (VZV) related arterial ischemic stroke (AIS) has been described in literature in pediatric age. However, the long-term course of post-VZV vasculopathy need to be inquired: clear information about prevalence of recurrence and severity of clinical outcome are lacking, even if a favorable evolution was initially described, and therapeutic protocols are not currently standardized. Objectives: We aimed to describe the clinical, laboratory and neuroradiologic features of children affected by AIS due to post-VZV referred to our Institute and to present our experience in their therapeutic management. Methods: We selected 22 pediatric patients (6 females) with AIS and a CNS confirmed VZV reactivation and/or with a VZV history in the previous 12 months. Other causes of pediatric stroke (systemic disease, cardiac disease, trauma, major thrombophilia) were excluded. Clinical, neuroimaging, laboratory and treatment data were reviewed, focusing on pediatric score outcome measure (PSOM) and executive functions final outcome. Results: Average age of AIS onset, VZV primary infection and interval between infection and AIS were: 4 years 10mo (range: 1 year and 8 mo-9 years and 11 months), 4 years and 5 months (range 8 months-9.4 years), and 7 months (range 10days-34 months), respectively. The AIS involved the nucleo-capsular region in 18 cases, the cerebral cortex in 9 cases, the thalamus in 4 cases, and the pons in 3 subjects. Seventeen patients had inflammatory focal cerebral arteriopathy (iFCA). Virological confirmation (VZV-DNA or anti-VZV IgG in the cerebrospinal fluid) was obtained in 11 patients. Three patients were treated with trombectomy and one with rTPA. Thirteen patients were treated with antiviral agents associated with steroids in 8 cases, with different administration schedules. Only in one case steroid treatment was given without association with antiviral agents. One patient received a short course of steroid and antiviral treatment at the time of the stroke and then a more prolonged course after six months at the time of the virological diagnosis. Prophylactic antiaggregants were administered to all patients. Mean follow-up was 2 years and 5 months (range 6 mo -10 years) ; iFCA was persistent in 12 cases and transient in 5 subjects. Four patients presented a recurrence of post VZV arteriopathy, two of them presenting new stroke events. Twelve patients presented a variable motor deficit at last follow up. The mean PSOM score of the cohort at the last visit was 1 (range 0-2). Executive functions were evaluated at last follow up in twelve patients, showing no deficit in seven patients, a mild deficit in two patients and a severe deficit in the last three. Conclusion: Albeit a favourable evolution was initially described, our experience suggests that VZV-related AIS may result in persistent FCA and significant neurological impairment in the majority of cases. Therapeutic approach, particularly involving steroid administration, still need to be validated. Introduction: IgA vasculitis/ Henoch Schönlein Purpura (IgAV/HSP) is the most common vasculitis of childhood and renal involvement is the most serious long-term complication. A better understanding of the pathophysiology of the progression to kidney disease is required for better treatment to be achieved and current biomarkers of Ig A vasculitis with nephritis (IgAVN) lack the predictive value.

Objectives: In this study, an untargeted metabolomics approach was performed to reveal the underlying molecular mechanism of disease pathogenesis and to find potential biomarkers of plasma samples from patients with IgAV and IgAVN.Methods: IgAV was diagnosed according to the Ankara criteria in 2008 (1). Forty-five patients, including 39 active IgAV patients (H), 6 IgAVN (N), and 6 age-and gender-matched healthy controls (C), were enrolled in the study. Plasma samples from subjects were collected on the same day of IgAV(HSP) diagnosis and before steroid or other immunosuppressive treatment initiated. This study has utilized liquid chromatography-mass spectrometry (LC-MS/ Q-TOF) to investigate the alterations in plasma metabolomic profiles. Three separate pools, health controls, active IgAV , and IgAVN were created. Peak picking, grouping, and comparison parts were performed (metabolite profiling) via XCMS (https://xcmsonline.scripps.edu/) software. Results: Totally 2618 peaks were detected for group H, N and C. Among them 355 peaks were found to be statistically significant and reliable (p< 0.05) and 155 of these peaks were found to be changed (fold change >1.5) between the groups C and H. On the other hand, 66 peaks were found to be changed (fold change >1.5) between the groups H and N. The number of the peaks on the intersection of the peaks found to be changed between the groups (C and H) and (H and N) was 39. Based on putative identification results, 15 peaks were matched with 11 metabolites. We found an up-regulated level of DHAP(18:0), prostaglandin D2/I2, 5methyltetrahydrofolic acid, porphobilinogen and N-Acetyl-4-O-acetylneuraminic acid/N-Acetyl-7-O-acetylneuraminic acid, 5-Aminopentanamide /5-Aminopentanoic acid, Glycocholic acid, Saccharopine, N2-Succinyl-L-ornithine, gamma Tocopherol, and Galactosylsphingosine /Glucosylsphingosine in IgAV patients.

In conclusion, we have identified a number of metabolites that may be associated with the pathogenesis of IgAV. We also suggest that DHAP (18:0), prostaglandin D2/I2, porphobilinogen, 5-methyltetrahydrofolic acid and N-Acetyl-4-Oacetylneuraminic acid/N-Acetyl-7-O-acetylneuraminic acid may serve as biomarkers for predicting kidney disease since they were increased only in the patients who developed renal involvement at follow-up. Children were divided into four groups: those with JIA who didn't receive MTX yet (group 1); those who received MTX less than one gram during whole treatment (group 2); those who received MTX from 1 to 3 grams (group 3); children, received more than 3 grams of MTX (group 4).

The autoimmune inflammatory process in JIA can cause formation of pathological changes in the liver, even before the start of treatment. It is confirmed by a statistically significant correlation of BFGF level in 1st group with liver steatosis according to ultrasound examination (r = 0.8) and the level of C-reactive protein (r = 0.7). This indicates a close relationship between the intensity of the inflammatory process and collagen synthesis activation, which can further provoke liver fibrosis. Alterative processes in the liver associated with autoimmune inflammation, as evidenced by the presence of a positive correlation between the level of ALT and BFGF (r = 0.5). Upon reaching MTX dose 1 gram and 3 grams, it is possible that compensatory processes in the liver are triggered, as evidenced by the negative correlation between the content of BFGF and HGF (r = -0.6).Conclusion: The use of modern markers with routine laboratory and instrumental studies is appropriate for the timely determination of the risks of developing irreversible pathological changes in the liver during JIA treatment with MTX. Objectives: The aim of our study is to evaluate the efficacy of (IAG) injections in hip in children with (JIA) and to assess the factors predicting the improvement of this management.

Methods: This is a retrospective study, between 2006 and 2009, including patients with JIA diagnosed according to the ILAR criteria. The socio-demographic data were collected as well as the parameters of the disease. The activity was evaluated by JADAS. The functional impact was assessed by the Lequesne score. The treatments taken have been specified as well as the infiltrations received. The improvement after infiltration was assessed by JADAS and Lequesne score.Results: Fourteen patients were included, with mean age 17.21 +6.8 . The mean age at the onset of symptoms was 11 +0.5 [3] [4] [5] [6] [7] [8] [9] [10] [11] [12] [13] [14] [15] . Subtypes of JIA according to The ILAR were: enthesitis-related arthritis in 7 cases, seropositive polyarticular JIA in 2 cases, seronegative polyarticular JIA in 2 cases, oligoarticular JIA in 2 cases and juvenile psoriatic arthritis in one case. All the patients had hip arthritis, inaugural in 90% of the cases. Of these, 92.8% had a flexion deformity and lower limb inequality. The average Lequesne index was 8.5 +4.6. The treatments taken were Methotrexate in 57.14% of the cases, Sulfasalazine in 14.28% of the cases, and the combination of the two in 21.4% of the cases. Eleven patients underwent hip infiltration, and three of them required more than one. Eighty one percent improved thereafter. The number of infiltrations was not statistically associated with the Lequesne index (p = 0.069). Improvement after infiltration was negatively associated with the prior existence of an inequality of the lower limbs (p = 0.04). The existence of a flexion deformity was not associated with good results after infiltration (p = 0.476, r=-0,624). Ten patients (90%) among those who had an infiltration did not have to resort to surgery. Conclusion: IAG injection is an adjunct therapy in AJI with hip involvement offering a good results and delay surgery in the majority of cases. The presence of lower limb inequality is associated with less improvement of IAG. Conclusion: Synovial rice bodies are rarely described in juvenile idiopathic arthritis, even less at disease onset. Their presence has not been associated to a worse disease prognosis or joint outcome but awareness of the existence of this particular form of intraarticular loose bodies may encourage the clinician to use lower gauge needle during arthrocentesis procedure; this can prevent arthroscopy, as occurred in our case 1. Arthroscopy may be necessary in some cases to achieve full drainage of the joint. In our series the duration of arthritis correlated with the size of rice bodies and the number and agressiveness of procedures needed to evacuate them. Objectives: We described a case of liver involvement in SLE presenting with emphasis on the differential diagnosis with autoimmune hepatitis. Methods: case report study Results: : An 8-year-old female patient was referred to the Rheumatology clinic with complaints icteric sclera for 10 months anorexia, malaise, pain in the both knees, ankles joints and both wrists accompanied by swelling, and remarkable motion limitations. Laboratory revealed T bilirubin 4.9 mainly direct 3.9 with elevated liver enzymes GOT 401, GPT 189, ALKP 520, high Glutamyl transpeptidase 56U\L her WBC 7.4 HGB 10, PTL 317, except very high ESR 105ml\hr, CRP was positive 190mg\dl, viral screen (HCV, HBSAg, HIV) was normal, serology tests ANA was positive with high titer 1280, anti ds-DNA AB was positive 320, anti-Sm was negative, ANTI LKM1 antibodies negative, anti smooth muscle AB negative soluble liver antigen were negative, antimitohondrial AB( M1,M2,M3). ultrasound abdomen revealed mild enlarged spleen, abnormal diffused increased liver echogenicity with early stage of liver cirrhosis treated her by fresh frozen plasma 5 times, ViT k 10mg once\ day then was referred to rheumatology clinic regarding her serology tests & developed arthritis of her joints suspected PSLE! She was performed liver biopsy showed lesions necrotic inflammatory portal and lobular severe in eosinophilic polynuclear with cirrhosis evoking a syndrome of overlap associating a primary biliary cirrhosis and an autoimmune hepatitis. Laboratory data revealed liver dysfunction and liver biopsy Suggesting autoimmune hepatitis, and she underwent treatment for hepatitis (prednisolone with azathioprine), Urosdoxycholic acid with fat-soluble vitamins K, D&A, E. However, with the elimination of jaundice and decreased hepatic enzyme levels, the prednisolone dose was tapered within 2 months and stopped before they were referred to Rheumatology clinic. On her review of systems, she has malar rash, generalized fatigability. On physical examination, we found malar rash, levidoreticularis of her skin, swelling and limitation of movement in the knees, ankles, wrists joints. There was hepatosplenomegaly. Laboratory data revealed liver treatment for hepatitis, ANA still high titer 1:1280, Antids DAN positive with titer 307 IU\ml, antiSMA was negative .WBC 4.5, HGB 11.8, PLT 268, ESR 68ml\hr, Her ultrasound abdomen: revealed slightly heterogeneous liver with coarse echotexture without focal lesion with liver span 14 cm.These paraclinical results together with the clinical findings strongly suggested systemic lupus erythematosus (SLE) as the definitive diagnosis. Indeed, in this case, AIH was associated with SLE, prednisolone orally for 2 months, after that dose was tapered and continued, rapid clinical improvement in arthritis, malaise, and general condition. Azathioprine was continued. In addition, daily hydroxycholoquine sulfate

Overlapping of SLE and AIH should be suspected when AIH patients present with a malar or other skin rash. The prompt diagnosis and adjustment of further treatment plans can improve disease outcomes and prevent liver disease progression. Introduction: Juvenile systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multi-visceral involvement with an unpredictable prognosis. The diagnosis is usually made in young women aged between 20 to 40years, however, it can affect people at any age and it is classified as a juvenile illness when it starts before the age of 16. Objectives: We are reporting the epidemical, clinical, therapeutical and evolutional characteristics of a series done in the pediatric Pole in setif with 13 girls and 1 boy.

Methods: The average age of onset is 13 years. The average time limits of the diagnosis is 7 months. The clinical features is done with cutaneous, articular manifestations and fever respectively in 100% 71% and 57% of the cases ,followed by kidney damage in 42% of the cases , the cardiac, pulmonary and ophthalmological participations are reported with low percentage. Haematological involvement was detected in 85% of the patients and the inflammatory syndrome was almost constant. A positive titer of anti-nuclear antibodies and anti-DNA is objectified, as well as a reduction in the complement rate. Antibodies anti GP 2 and anti cardiolopine are positive in 57% of cases. Kidney damage was diagnosed in 42% of the cases , and only one case of overlap syndrome with dermatomyosits was reported. Concerning the neurological form it was present in only one addolecent girl ,and only one case of familial lupus.Results: The diagnosis is based on the classification of the American College of rheumatology (ACR) 1982 revised on 1997 and the new criteria SLICC"Systemic Lupus International Collaborating Clinics" . The clinical characteristics of our series relies on global data of literature with the Predominance of Cutaneous and articular involvement. with however some specific characteristics which are individualized by a more advanced age of onset, 13 years on average in our study versus 10 years and 12 years, the rarity of familial forms (1 case), a lower percentage of kidney damage (42% versus 63% and 80%).The therapeutic management was based on corticosteroid therapy and Hydroxychloroquine in the majority of cases, the use of immunosuppressants has been reserved for severe forms. Conclusion: Lupus is an autoimmune disease with protean clinical manifestations, the prognosis of which is dominated by renal, neurological and thrombotic disorders. Cortisonic treatments and immunosuppressants have significantly improved the prognosis for life . Trial registration identifying number: Lupus is an autoimmune disease with protean clinical manifestations, the prognosis of which is dominated by renal, neurological and thrombotic disorders. Cortisonic treatments and immunosuppressants have significantly improved the prognosis for life . onset of inactive and active oligo-JA were not significantly differ. The analysis revealed a correlation between a short phase of beneficial effect after is-IAI of TA and risk of activity disease (with an inactive phase of arthritis less than 3 months, the risk activity was OR = 2.09, p <0.001; with an inactive phase less than 2 months -OR = 8.9, p < 0.001). RTX was administered to patients who had received high-dose CS with 2-3 DMARDS; in all cases combined pulse therapy CS № 2-10 was preliminarily used. RTX 500 mg № 2 was applied after 6mo-2y from the debut of the disease. In all 5 cases, its use led to clinical improvement after 1-5 mo with normalization of laboratory activity indicators, in 4 cases a decrease in the level of B cells to 0-0.56 in μl was noted (3 with agammaglobulinemia). After 2 months 3 patients had severe infectious complications, 2 of them ended fatally. 2 another patients had a second stroke. The 1st patient survived, had a kidney allotransplantation, there is no disease activity. The 2nd patient, in connection with the development of the demyelinating process of CNS, attempted to continue therapy using golimumab with IVIG. It led to an increase in the infectious syndrome, therefore, we decided to refrain from continuing with iTNF as well. The patient died after 2 years from the administration of RTX due to the progression of neurological disorders. 2 cases with auto-inflammatory syndromes were: chronic infantile neurologic cutaneous and articular syndrome received TCZ; it was unsuccessful (hyperthermia and rash persisted, eye lesions progressed, there were no increase in height), later switched to anakinra. Family mediterranean fever, received adalimumab (ADA). The 1-year-course of ADA leaded to the disappearance of articular and abdominal syndrome while maintaining persistent increased levels of ESR and CRP and periodic fever. The use of TCZ in 2 patients with SSD was more successful. The first patient received it subcutaneously for 1 year, CS&DMARDs (3 were used) had already been canceled, lung and kidney lesions were contained, blood pressure normalized, EScSG-AI decreased from 7 to 1, MRSS decreased from 18 to 14. In the second case, the patient received TCZ for 6 months i/v, decrease of EScSG-AI 6.5 to 1, MRSS 33 to 21 were noted, the dose of CS was halved, he also continued treatment with cyclophosphamide. We introduce a 13-year-old girl patient who has been admitted to our clinic with suspicion of an erythema nodosum. She had painful subcutaneous nodules for 4 weeks, especially on the lower extremities and her face. Macroscopically, central necrotizing skin rashes could be seen. She had frank arthritis of both knee and ankle joints. The comprehensive serological diagnosis (including hepatitis serology and anti-streptolysin titer) were normal except for a slight increase in CRP 0,9 mg/dl and ESR 36 mm/h. The patient also complained of abdominal pain and bloody stools. Calprotectin was 3613 μg/g. A Gastro-coloscopy revealed a small mariske and a minimal inflammation of the Ileocecal valve, without signs of vasculitis or chronic bowel disease. A skin biopsy revealed leukocytoclastic vasculitis of the small arteries. Angiography of the intestinal arteries was rejected by the family. Initially we started a treatment with methylprednisolone pulses followed by oral prednisolone. The patient showed a very good response with quick resolution of the skin symptoms and abdominal pain. The medication could be quickly tapered and discontinued at full remission after one month Results: PAN is classified as a cutaneous PAN (cPAN) when there are exclusive skin manifestations, besides arthralgia or arthritis. A systemic PAN must be diagnosed with the involvement of internal organs. However, cutaneous PAN may evolve into systemic PAN. In our patient, the skin and joints were primarily affected. If the existing gastrointestinal complaints are part of a systemic PAN or chronic bowel disease could not be cleared yet, due to refusal of further investigations.

Conclusion: cPAN must be considered as a suspected diagnosis in patients with necrotizing skin nodules. As transition of the cutaneous into the systemic form cannot be predicted regular monitoring is mandatory.

Introduction: Prevalence of Behcet's disease in children is not known, but is probably very low. Extra-pulmonary large vessel arteritis in these cases is even rarer as a presenting manifestation. Objectives: To report two cases of paediatric extrapulmonary large vessel arteritis with a 'Behcet like disease'. Methods: We present case reports of two cases who presented to paediatric rheumatology OPD to our department. Ms. F, a 16 year old girl was referred to us with history of short duration of fever, generalized lymphadenopathy, neutrophilic leucocytosis, thrombocytosis, hyperglobulinemia and high inflammatory markers. On detailed history and examination she was found to have a healed palatal ulcer and her maternal aunt was found to have a history of recurrent oral ulcer, genital ulcer and enthesitis. Patient's Montoux test was positive but the gene expert for MTB was negative. MD-CT showed a circumferential thickening of aorta, subclavian and bilateral renal artery with stenosis at origin of both renal arteries indicating a vasculitis. Few necrotic nodes were also noted in lungs. Lymph node biopsy suggested a reactive hyperplasia. Tissue typing showed presence of HLA B 44, B 51. She improved clinically with oral Prednisolone and Mycophenolate Mofetil and had no recurrence till her recent follow up visit. Second case, master FKN an 11 year old child was referred to us with a background of 2 week history of fever, non migratory arthritis, raised inflammatory markers and a symptomatic severe aortic regurgitation with pandiastolic flow reversal on 2D echo. His evaluation showed negative Montoux, normal IGG4 levels and HLA B35 B 51 on tissue typing. His aortic wall thickness resolved with 1 mg/kg oral Prednisolone and Mycofenolate mofetil. Results: Both these cases have features similar to Behcet's disease. These cases do not fulfil ISG, ICBD 2014 or ICBD criteria for pediatric Behcet's disease. However, the aortitis and other clinical features responded well to the treatment in both cases.Conclusion: Paediatric case with extra-pulmonary Large vessel arteritis that do not meet criteria for Behcet's disease but have specific clinical or laboratory features do respond well to immunosuppression. Therefore, after ruling out other causes of the large vessel vasculitis, a possibility of form fruste of Behcet's disease should be under consideration.

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These abstracts have been published as part of Pediatric Rheumatology, Volume 18, Supplement 2, 2020: Proceedings of the 26th European Paediatric Rheumatology Congress (PReS 2020). The full contents of the supplement are available at https://ped-rheum. biomedcentral.com/articles/supplements/volume-18-supplement-2. Please note that this is part 2 of 2.