key: cord-335597-anrzcsrt authors: nan title: 44. Jahrestagung der Österreichischen Gesellschaft für Pneumologie date: 2020-10-26 journal: Wien Klin Wochenschr DOI: 10.1007/s00508-020-01745-3 sha: doc_id: 335597 cord_uid: anrzcsrt nan Patientencharakteristik, Anamnese, Symptome: Eine 40-jährige Patientin wurde nach plötzlichem Auftreten von Dyspnoe, Tachypnoe und rechtsthorakalen Schmerzen mit Ausstrahlung in den rechten Oberbauch vorstellig. Die Anamnese bezüglich Traumas oder chronischen Vorerkrankungen war unauffällig. Diagnostik und Diagnose: Die Blutgasanalyse zeigte eine Hypoxie. Laborchemisch fand sich lediglich eine isolierte, milde Leukozytose. Die Thoraxauskultation ergab rechtsseitig verminderte Atemgeräusche. Die Computertomografie des Thorax wies einen rechtsseitigen hinteren Zwerchfelldefekt mit intrathorakaler Hernierung des Kolon ascendens und rechtsseitigen Pneumothorax mit Mediastinalshift nach links aus. Bildgebend bestand mithin der Verdacht auf eine Kolonperforation und eine Bochdalek-Hernie. Differentialdiagnostik: Obwohl Bochdalek-Hernien bei Erwachsenen selten sind, müssen sie differentialdiagnostisch bei Patienten mit Dyspnoe berücksichtigt werden. Eine intrathorakale Kontamination nach bakterieller Translokation oder Hohlorganperforation kann zur Entwicklung von Pleuraempyemen führen. Die sorgfältige intraoperative Lavage und Drainage der Thoraxhöhle hat deshalb in diesen Fällen große Bedeutung. Therapie: Es erfolgte eine Notfalllaparotomie, welche die Diagnose bestätigte. Ein 2 cm langer Zwerchfelldefekt mit inkarzeriertem und perforiertem Kolon ascendens wurde erkannt. Unter Erweiterung der Bruchpforte konnte das Kolon nach intraabdominell reponiert werden. Aufgrund von ischämischen Veränderungen sowie der Kolonperforation wurde eine rechtsseitige Hemikolektomie mit Anlage einer Seit-zu-Seit Ileotransversostomie erforderlich. Nach ausgedehnter abdomineller und transdiaphragmaler thorakaler Lavage wurde eine Bülaudrainage platziert, Zwerchfelldefekt und Bauchdecke wurden mittels Naht verschlossen. Am 13. postoperativen Tag wurde aufgrund eines rechtsseitigen Pleuraempyems nach diagnostischer VATS eine anterolaterale Thorakotomie mit anschließender Pleuradekortikation zur Sanierung erforderlich. Die Patientin erholte sich letztlich gut und wurde am 12. postoperativen Tag nach Hause entlassen. Patientencharakteristik, Anamnese, Symptome: Im Juli 2020 wird eine 62-jährige Patientin vom niedergelassenen Pneumologen an unsere Abteilung überwiesen. Die Aufnahme erfolgt aufgrund einer vor ca. zwei Wochen suspizierten Pneumonie, welche mit Amoxicillin-Clavulansäure behandelt wurde. Im Aufnahmegespräch berichtet die Patientin über deutliche Abgeschlagenheit und Belastungsdyspnoe (Stiegen steigen) bei bisher altersentsprechend sehr guter Leistungsfähigkeit. In der Anamnese sind eine atypische Pneumonie vor einem Jahr, eine primär biliäre Cholangitis (ED 2019 im Rahmen der Pneumonie) und eine substituierte Hypothyreose auffällig. In den von uns initial erhobenen Laborbefunden zeigen sich moderat erhöhte Entzündungsparameter (CRP 5,79 mg/ dl, Leukozyten 15,1 G/L), woraufhin die antibiotische Therapie auf Azithromycin 500 mg umgestellt wird. Eine durchgeführte Computertomographie des Thorax zeigt bipulmonale fleckige Milchglastrübungen, peribronchovaskuläre Verdichtungen mit teils positivem Pneumobronchogramm und hiläre sowie mediastinale Lymphadenopathie. Zur Abklärung der dementsprechend im Raum stehenden Differenzialdiagnosen einer Sarkoidose, Vaskulitis bzw. eines Lymphoms werden eine Bronchoskopie mit transbronchialer Lymphknotenbiopsie (LK11L, LK11R, LK7 und LK4R) und detaillierte laborchemische Untersuchungen durchgeführt. Diagnostik und Diagnose: Im Verlauf kommt es zu einer Verschlechterung der Nierenfunktion (Creatinin 1,77 mg/dl) und einer Proteinurie (1,4 g/l), ansteigenden Entzündungsparametern (CRP, Leukozyten, BSG) und einem positiven Ergebnis für MPO-ANCA (27 IU/ml). Die histopathologische Aufarbeitung der Lymphknotenbiopsien zeigt ausgeprägte reaktive Zell-und Kernveränderungen ohne Hinweis für eine Infiltration mit malignen Zellen. Die erhobenen Befunde erhärten den Verdacht auf eine MPA-ANCA-assoziierte Vaskulitis mit pulmonaler und renaler Beteiligung. Die Patientin wird an die Universitätsklinik für Nephrologie transferiert, wo komplikationslos eine Nierenbiopsie durchgeführt wird. Hierbei zeigt sich das histologische Bild einer Pauci-immunen Glomerulonephritis mit Halbmondbildungen und Schlingennekrosen in 50 % der Glomeruli, vereinbar mit der Diagnose einer MPO-ANCAassoziierte Vaskulitis. Therapie: Nach entsprechender Aufklärung der Patientin beginnen wir mit der Therapie mit Rituximab 1000 mg (aktuell zwei Gaben erhalten, dritte Gabe geplant) sowie Methylpred-Therapie: Im Rahmen einer rechtsseitigen Thorakotomie erfolgte eine offene Wedge-Resektion des Oberlappen sowie Mittellappens rechts, wobei sich intraoperativ histologisch der Verdacht einer Lymphominfiltration ergab. Die Fistulierung zur Speiseröhre wurde mittels Vena azygos-Patch gedeckt und übernäht. Eine protektive endoluminale VAC-Anlage konnte bereits am 4. postoperativen Tag entfernt werden. Histologisch bestätige sich ein aggressives Non-Hodgkin-Lymphom der B-Zellreihe, speziell eines diffusen großzelligen B-Zell-Lymphoms (zentroblastisch-polymorph), Nicht-Keimzentrumstyp nach Hans-Klassifikator. Von onkologischer Seite wurde einer Therapie mittels R-CHOP initiiert bei postoperativ chirurgisch unauffälligem Status. Allergie mit Biss Background: Lung transplantation is the ultimate treatment option for patients with end-stage respiratory diseases but bears the highest mortality rate among all solid organ transplantations due to chronic lung allograft dysfunction (CLAD). The mechanisms leading to CLAD remain elusive due to insufficient understanding of the complex post-transplant adaptation processes. Here, we aimed to better understand the processes preceding CLAD, and investigate their association with future changes in allograft function. Methods: We performed an exploratory cohort study in 78 patients, including broncho-alveolar lavage samples from lung donors and recipients (after transplantation). We analyzed the alveolar microbiome using 16S rRNA sequencing, the cellular composition using flow-cytometry, and conducted metabolome and lipidome profiling. Results: We established distinct temporal dynamics for each of the analyzed data sets. Comparing matched donor and recipient samples, we revealed that recipient-specific as well as environmental factors, rather than the donor microbiome, shape the long-term lung microbiome. We further discovered that the abundance of certain bacterial strains correlated with underlying lung diseases even after transplantation. A decline in forced expiratory volume during the first second (FEV1) is a major characteristic of lung allograft dysfunction in transplant recipients. By using a machine learning approach, we could accurately predict future changes in FEV1 from our multi-omics data, whereby microbial profiles showed a particularly high predictive power. Conclusions: Broncho-alveolar microbiome, cellular composition, metabolome and lipidome show specific temporal dynamics after lung transplantation. The lung microbiome can predict future changes in lung function with high precision. über eine anterolaterale Thorakotomie rechts im 6. ICR inklusive adäquate onkologische Lymphadenektomie Position 4 rechts bis 11i The authors marked with an asterisk (*) are the corresponding authors. abstracts ÖGP Conclusions: Patients with COPD are insufficiently evaluated for CAD due to overlapping symptoms. Current CAD risk scores for stable chest pain appear inappropriate for patients with COPD. Background: Oncologic patients are regarded the population most at risk of developing a severe course of COVID-19 due to the fact that malignant diseases and chemotherapy often weaken the immune system. In the face of the ongoing SARS-CoV-2 pandemic, how particular patients deal with this infection remains an important question. In the period between the 15th and 26th of April 2020, a total of 1227 patients were tested in one of seven oncologic outpatient clinics for SARS-CoV-2, regardless of symptoms, employing RT-qPCR using BGI Real-time fluorescent RT-PCR kit for detecting 2019-nCoV2 on Applied Bioscience ABI7500 instruments. Results: Of 1227 patients, seventy-eight (6.4 %) were tested positive of SARS-CoV-2. Only one of the patients who tested positive developed a severe form of COVID-19 with pneumonia (CURB-65 score of 2), and two patients showed mild symptoms. Fourteen out of 75 asymptomatic but positively tested patients received chemotherapy or chemo-immunotherapy according to their regular therapy algorithm (+/-4 weeks of SARS-CoV-2 test), and 48 of 78 (61.5 %) positive tested patients received glucocorticoids as co-medication. None of the asymptomatic P02 Current symptom-based risk scores for stable coronary artery disease evaluation are not applicable in COPD patients Background: Cardiovascular diseases are arguably the most important comorbidity in patients with chronic obstructive pulmonary disease (COPD). Despite an increased prevalence of coronary artery disease (CAD) in COPD patients, there are no dedicated diagnostic recommendations. We investigated whether COPD patients receive adequate primary evaluation of CAD despite overlapping symptoms. Methods: 302 patients with COPD, who underwent invasive coronary angiography (ICA), were retrospectively matched (for age, BMI and cardiovascular risk factors) with 302 patients without functional lung diseases. Quality and onset of symptoms prior to ICA were documented and individual patients' pre-test probabilities according to ESC guidelines were calculated. Endpoints were delay of ICA referral after symptom onset and clinical outcome, defined as subsequent revascularization. Results: Mean delay between symptom onset and ICA was 19.9 ± 22.0 months in COPD patients compared to 8.3 ± 12.7 months in the control group (p < 0.0001). COPD patients had a lower rate of typical chest pain (25.2 % vs. 38.1 %, p = 0.0009), and dyspnoea only (18.2 % vs. 26.8 %, p = 0.015). Sub-analysis of GOLD grades revealed an incremental delay with increasing COPD severity: GOLD 1: 16.1 ± 17.3 months; GOLD 2: 17.6 ± 22.1 months; GOLD 3: 20.1 ± 21.3 months and GOLD 4: 24.2 ± 23.4 months. Furthermore, the revascularization rate increased with higher pre-test probability for the control group, but not for patients with COPD GOLD 1-4. abstracts Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by severe pulmonary artery hypertension and presence of sleep-disordered breathing (SDB) with associated hypoxemia which could further contribute to the severity of hypertension adversely affecting the outcome. Limited data are available on the prevalence of SDB in CTEPH and so far, the effect of balloon pulmonary angioplasty (BPA) on SDB has not been evaluated. We hypothesized that subjects with CTEPH have a high prevalence of SDB, both obstructive and central sleep apnea with associated hypoxemia, which could improve with BPA. Methods: 33 consecutive patients with CTEPH underwent treatment-naïve and post-BPA polygraphy (nasal-pressure-sensor, thermistor, thoracoabdominal-excursion-sensors, pulse oximeter; Alice PDx, Philipps®) and hemodynamic and echocardiographic assessments. Results: Before BPA, prevalence of SDB (defined as an apnea-hypopnea index (AHI) >5 per hour) was 75 %: 8 patients infected patients showed unexpected complications due to the SARS-CoV-2 infection during the cancer treatment. Conclusions: These data clearly contrast the view that patients with an oncologic disease are particularly vulnerable to SARS-CoV-2 and suggest that compromising therapies could be continued or started despite the ongoing pandemic. Moreover, the relatively low appearance of symptoms due to COVID-19 among patients on chemotherapy and other immunosuppressive co-medication like glucocorticoids indicate that suppressing the response capacity of the immune system reduces disease severity. Background: For the further crisis management of the corona pandemic and the socio-economic impact on society, a strategy that allows selective isolation measures is particularly important. So far, it has been assumed that patients suffering from COVID-19 develop antibodies that provide immunity and are thus protected from a reinfection with SARS-CoV-2. This also forms the basis of the assumption that rapid vaccine development will lead to rapid control of the pandemic. In the present study, we analyzed the antibody development of 77 oncology patients 14 days after positive RT-qPCR testing for SARS CoV2. Methods: RT-qPCR and anti-SARS-CoV2-antibody methods from BGI (MGIEasy Magnetic Beads Virus DNA/RNA Extraction Kit) and Roche (Elecsys Anti-SARS-CoV-2 immunoassay) were used, respectively, according to the manufacturers' specifications. Results: Surprisingly, in only 6 of 77 individuals with a confirmed history of COVID-19 antibody development was detected. Despite of multiple testing, these patients did not develop antibodies in subsequent tests. Conclusions: First analyses indicate that patients may benefit from inpatient PR after hospitalization due to COVID-19. Symptoms of dyspnea, cough, depression, and anxiety decreased significantly over the course of the PR, whereas quality of life significantly increased. PR could therefore play an important role in dealing with the pandemic. Follow-up assessments three and six months after the PR are currently ongoing. Background: Fatigue is among the most common symptoms in COVID-19 patients and about 50 % still suffer from persistent fatigue 2 months later [1] . Some studies discuss a possible link to obstructive sleep apnea syndrome (OSAS) [2] , however, no studies have been published in COVID-19 patients after discharge. Therefore, we examined COVID-19 patients for the presence of OSAS within 4 weeks after acute hospital discharge during inpatient pulmonary rehabilitation. Methods: From May until July 2020 we screened all eligible COVID-19 patients for OSAS using polygraphy. If the screening revealed an Apnea-Hypopnea Index (AHI) of ≥ 15, further diagnostics using polysomnography were conducted. Furthermore, we assessed the sleepiness using the Epworth Sleepiness Scale (ESS) and the Body-Mass-Index (BMI). Results: 37 patients were eligible for the study, of which 24 were willing to participate. Mean age was 56.4 (range: 33-83), 24 % were female, and mean BMI was 31.8 (range: 22.2-53.3). Only four patients had an AHI <5, whereas eleven fulfilled the criteria for an at least moderately severe OSAS (AHI ≥15). The AHI was significantly correlated with the BMI (r = 0.44, p < 0.05) but not with the ESS (r = -0.09, p = 0.71). A positive airway pressure therapy was indicated in eight patients (33 %); five agreed to the therapy (20 %). Conclusions: According to our data, rates of OSAS are extraordinarily high in patients after hospitalization due to COVID-19. This could be an explanation for frequently mentioned symptoms of tiredness and attention deficit. Yet, further studies are needed to examine a possible causal association and correlations to other common symptoms, such as cognitive impairment or sleep disturbances. (24 %) without SDB, 18 (54 %) with predominantly obstructive sleep apnea (OSA; AHI = 26), and 7 (21 %) with predominantly central sleep apnea (CSA; AHI = 41). OSA was associated with male-gender, obesity and overnight fluid-shifts, whereas CSA with worse right ventricular end-diastolic diameter. Patients with SDB had significantly higher oxygen-desaturation index (ODI) and tendency for worse desaturation than those without SDB. After BPA, mean AHI and ODI decreased by 45 % (p = 0.004) and 28 % (p = 0.001). In OSA patients, AHI decreased from 26 to 19 (p = 0.088) and in CSA patients from 41 to 21 (p = 0.011). Along with improvement in SDB, nocturnal desaturation decreased (time-below-90 % from 50 % to 43 % of time-in-bed, p = 0.037). Conclusions: This is the first study of the effects of BPA on SDB in CTEPH. We found high prevalence of SDB, both OSA and CSA, in consecutive subjects underdoing BPA, and report that BPA significantly improved sleep-disordered breathing and nocturnal desaturations. Future randomized controlled trials are needed to determine if effective treatment of SDB improves central hemodynamics, morbidity and mortality of patients with CTEPH. Pulmonary rehabilitation following COVID-19 -First short-term results regarding symptoms, quality of life, and psychological burden of disease Background: Even though many studies have been published on COVID-19 within the last months, little is known about the results of pulmonary rehabilitation (PR) following a severe infection. Therefore, the current study examines the changes of wellbeing through inpatient PR after COVID-19. Methods: We surveyed patients at the beginning (T1) and the end (T2) of inpatient PR following hospitalization due to COVID-19. We assessed respiratory symptoms (dyspnea, cough, and phlegm expectoration) and pain with symptom rating scales, fatigue with the Brief Fatigue Inventory (BFI), quality of life with the EuroQol-Questionnaire (EQ-5D-SL), and symptoms of depression and anxiety with the Patient Health Questionnaire (PHQ-D). Results: From the beginning of May until the end of June 2020, 31 patients were eligible, of which 25 patients could be included in the study and completed all T1 and T2 assessments (Mean age: 58.1; 38.9 % female; 52.8 % after invasive ventilation). At T1 the participants were heavily burdened and dyspnea on exertion was by far the most common and burdensome impairment. Over the course of the PR (mean treatment duration: 26 days; range: 21-35 days), the data revealed improvements in all mentioned outcomes. Dependent samples t-tests revealed statistical significance in all variables, except for pain (p = 0.058) and phlegm expectoration (p = 0.258). Effect sizes ranged from small (d = 0.48, p < 0.01 for dyspnea at rest) to large abstracts Background: Patients with repaired congenital diaphragmatic hernia (CDH) often suffer from obstructive airway disease. Nitrogen multiple breath washout (N2-MBW) is a sensitive method to detect ventilation inhomogeneity and peripheral airway pathology with higher sensitivity than conventional spirometry. We set out to obtain detailed information about peripheral airway pathology by N2-MBW in addition to conventional lung function testing. Methods: We prospectively compared school-aged children following CDH repair and healthy controls using spirometry, body plethysmography and N2-MBW. Group analyses were made using T-test and Mann-Whitney-U Test, as appropriate. Matching criteria included age, gender and level of physical activity. Results: 13 (median [IQR] age 10 [8-13] years, f:m = 5:8) former patients and 11 matched healthy controls (9 [7-12] years, f:m = 4:7) were included. Mean lung clearance index (LCI) was highly similar in both groups (7.4 vs. 7.4; p = 0.893). Slope of conducting airways (Scond) was significantly higher (0.029 vs. 0.017; p = 0.032) in CDH patients. FEV1 (88 vs. 101 %pred; p = 0.009), MEF25 (72 vs. 108 %pred; p = 0.009), MEF50 (78 vs. 109 %pred; p = 0.002) and FEF25-75 (66 vs. 95 %pred; p = 0.003) were significantly lower in CDH patients. RV (133 vs. 85 %pred; p = 0.001), RV/TLC ratio (39 vs. 24 %pred; p = 0.001) and airway resistance (Reff ) (146 vs. 103 %pred; p = 0.003) were significantly higher in CDH patients, whereas there was no significant difference in TLC (99 vs. 106 %pred; p = 0.230) and FVC (100 vs. 102 %pred; p = 0.698). Three CDH patients had LCI and eight Scond values above the upper limit of normal (healthy controls: two and three, respectively). According to conventional lung function testing, 7/13 former patients showed an obstructive, none a restrictive pattern and six had normal lung function. FEV1% correlated significantly positively with MEF25%, MEF50% and FEF25-75 % and negatively with RV/TLC ratio. Conclusions: We found significant airway obstruction in both central and peripheral airways and hyperinflation in patients with congenital diaphragmatic hernia compared to healthy controls. Central sleep apnea in pacing-induced cardiomyopathy Background: Sleep disordered breathing, in particular central sleep apnea (CSA) is common in heart failure patients, but its role in pacing induced cardiomyopathy has not been studied yet. In this study entitled UPGRADE, we set out to evaluate the effect on sleep architecture and sleep disordered breathing in PICM patients receiving biventricular pacing. Methods: Presence of CSA was assessed by single-night polysomnography (PSG) in 54 PICM patients within one month after left ventricular lead implantation (with biventricular stimulation still not activated). CSA was diagnosed in half of patients (n = 27). Patients with moderate or severe CSA were randomized to cardiac resynchronisation therapy (CRT) versus right ventricular pacing (RVP) in a double-blinded cross-over design and re-scheduled for a follow up PSG 3-5 months, after repeated assessment of sleep and crossing-over another PSG was conducted 3-5 months later. Results: CRT led to a significant increase in left ventricular ejection fraction and significant reduction in left ventricular end systolic volumes and N-terminal pro brain natriuretic peptide plasma levels, whereas no significant effect was observed with ongoing RVP. CSA was significantly improved after 3.9 (3.2-4.4) months of CRT: apnea hypopnea index (AHI) decreased from 39.1 (32.1-54.0) events per hour at baseline to 22.2/h (10.9-36.7) by CRT (p < 0.001). Central apnea index decreased from 27.1/h (17.7-36.1) at baseline to 6.8/h (1.1-14.4) after CRT activation (p < 0.001). Ongoing RVP yielded only a minor improvement in AHI and central apnea index. Pre-existent CSA did not affect structural response rate and had no impact on mid-term follow up (median 2.8 years). Conclusions: CSA is highly prevalent in patients with PICM. CRT upgrading significantly improves CSA leading to a similar outcome in PICM patients without pre-existent CSA. UPGRADE is an investigator-initiated independent clinical trial, supported by the ÖNB Jubiläumsfondsprojekt Nr. 15974. This study was further supported by an unlimited scientific grant from the Boston Scientific Investigator Sponsored Research , named severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), was observed in Wuhan (China) for the first time and subsequently spread rapidly across the globe. The lung is the virus' primary target organ, but many other organs are affected, too. In consequence, therapy focuses on both, pulmonary and systemic symptoms. At present, there is no established pharmacological treatment for COVID-19 available, however, many studies are currently on their way. Treatment is based on local (i. e. inhalation) and systemic therapy, often in ventilated patients. Prerequisites for inhalation therapy are measures to prevent infection of health care personnel, use of adequate systems for drug administration and compounds suitable for pulmonary delivery. We reviewed publications on COVID-19 treatment for strategies for save inhalation therapy of ventilated/non-ventilated patients and compounds used in clinical studies. Strategies for inhalation administration differ in respect of disease severity and use of personnel protective equipment is essential. In mild-disease patients, asthma/COPD treatment is preferred by pMDIs/DPIs, if necessary. Jet/mesh nebulizers can be used with mouth pieces/nasal cannulas (no face masks to avoid aerosol spread) and one-way filters/valves. In ventilated patients, mesh nebulizers with filters should be used. Physical therapy/suctioning should not be combined with aerosol therapy (avoidance aerosol spread). Numerous compounds/biomolecules are under study for inhalation treatment of COVID-19, e. g. interferons (with/without systemic administration of antivirals, such as ribavirin, lopinavir, ritonavir), sargramostim (GM-CSF), aviptadil (synthetic vasoactive intestinal polypeptide), DAS181 (recombinant sialidase), PUL042 (immunostimulant, TLR 2/6/9 agonist), budesonide, nitrogen oxide and hydrogen. In summary, inhalation therapy is important for treatment of pulmonary symptoms of COVID-19. Strategies for pulmonary drug delivery differ in respect to disease severity (e. g. mild symptom patients vs. ventilated patients). Various pharmacological compounds/biomolecules are under study. However, up to now there is no established inhalation treatment of COVID-19. ual basis. Recent studies show that HS can improve health even in CF babies. Methods: The surveys provide information about acceptance, reason and frequency of 3-7 % HS application using a jet or vibrating membrane nebuliser (VMN; eFlow®rapid) in children ≤ 6 years. The online questionnaires address healthcare professionals in paediatric CF centres in the UK and German speaking countries (DACH). In the UK 21 and in DACH 17 paediatric CF centres participated in the survey. From both regions, a total of 54 healthcare professionals responsible for almost 1900 CF children ≤ 6 years of age responded. Results: Secretolysis by HS in children ≤ 6y is rated excellent, very good, or good by 81.1 % in UK and by 100 % in DACH. The tolerability is reported excellent to good by 67.1 % (UK)/100 % (DACH). In both surveys approx. 90 % of caregivers declare to use VMN in CF patients ≤ 6 y and less than 25 % in infants ≤ 2 y. Caregivers satisfaction regarding the ease of inhalation with VMN in infants ≤ 2 y is rated excellent to good by 70.5 % (UK)/59 % (DACH) and in the group 2-6y by 100 % (UK and DACH). Conclusions: The results of the survey reveal that in children ≤ 6 years nebulized HS for secretolytic therapy is general practice and well tolerated by this age group. They are consistent with the latest recommendations in the official CF guidelines for children, which confirm the benefits of using HS. In addition, the data also reflect the trend in the UK, where the annual report of the UK CF registry refers that the use of HS in young children is increasing every year. Expression patterns of heat shock protein 90 in patients with thymic epithelial tumors regarding the World Health Organization classification Background: Thymic epithelial tumors (TETs) are rare malignancies with unique association to the paraneoplastic syndrome myasthenia gravis (MG). Heat shock protein 90 (HSP90) harbour great potential as cancer biomarker and HSP90 inhibitors approach clinical cancer therapy. Methods: To investigate HSP90 tissue expression patterns, we analysed tumor tissues of completely resected TET patients (n = 101; 78 thymomas and 23 thymic carcinomas (TCs)), regular thymic tissue of six MG patients, and four patients without MG, Background: Pulmonary hemodynamics during exercise may help to identify early pulmonary vascular disease in systemic sclerosis (SSc). Whether they are of prognostic relevance in this subset of patients is unknown. We tested the association between pulmonary heamodynamics at rest and peak exercise with all-cause mortality in patients with SSc. Methods: All SSc patients with resting mPAP < 25 mm Hg and at least 1-year follow-up data who underwent symptomlimited exercise right heart catheterization between April 2005 and December 2018, were analyzed. Age-adjusted Cox-regression analyses were performed to assess the association between pulmonary hemodynamics and mortality. 76]) turned out as age-independent predictors of mortality. In contrast, resting pulmonary hemodynamics (mPAP, pulmonary arterial wedge pressure, CO, PVR and TPR) were not associated with age-adjusted mortality. Conclusions: In this study assessing the prognostic relevance of pulmonary exercise hemodynamics in patients with systemic sclerosis, PVR and TPR at peak exercise as well as mPAP/CO-slope and TPG/CO-slope turned out as age-independent predictors of all-cause mortality. Clinical survey with hypertonic saline (3-7%) and the eFlow®rapid in CF children below 6 years of age MNT: 100 % A: 60 % AB: 7 % B1: 11.1 % and B2: 11.5 B3: 100 % TC: 95.7 %). We detected HSP90 expression in centroblasts, but not centrocytes, of germinal centres in 100 % of MG patients with FTH. All lymphoid follicles of myasthenic patients expressed HSP90 protein. Hassall's Corpuscles showed no HSP90 expression in every tissue sample. We did not detect thymic HSP90 expression in four patients with regular thymic morphology or five patients with TTH. Conclusions: HSP90 expression data propose high potential for HSP90 as an additional immunohistochemical marker for MNT, WHO-B3 thymoma, and TC or as a possible candidate molecule for targeted therapy. Caution is warranted in TET patients with MG overexpressing HSP90. Later-line treatment with lorlatinib in ALKand ROS1-rearrangement-positive NSCLC: a retrospective, multicenter analysis Background: Anti-fibrotic medication is effective in progressive fibrosing interstitial lung diseases (ILD), but a subgroup of fibrotic ILD patients also benefits from immunomodulatory therapies. Additional to high-resolution computed tomography (HRCT), blood and broncho-alveolar lavage (BAL) biomarkers could help to identify such phenotypes. Methods: HRCT of 127 subsequent single-center ILDboard patients (mean age 65 (standard deviation 14) years, 65 % male), were evaluated for radiological findings considered noninflammatory (reticulation including honeycombing (RET), traction bronchiectasis (TBR), emphysema (EMP)) or active inflammatory (consolidations (CON), ground glass opacities (GGO), noduli (NDL), mosaic attenuation (MOS)) in 6 distinct lung regions. Each resulting score was further graded as minimal (0-1 regions involved), medium (2-4) and extensive (5-6). Associations between blood and BAL biomarkers and radiological finding scores were evaluated using Spearman correlation coefficients, Kruskal-Wallis tests were used for significance testing between the graded subgroups. Results: Blood neutrophil, lymphocyte and eosinophil fraction, neutrophil-lymphocyte ratio (NLR) and BAL lymphocyte fraction consistently showed opposite correlations for inflammatory versus non-inflammatory HRCT finding scores. Blood lymphocyte fraction significantly differed by the graded extent of GGO (p = 0.032) and CON (p = 0.027), eosinophil count by TBR (p = 0.006) and NLR by CON (p = 0.009). C-reactive protein significantly related to GGO (p = 0.023) and CON (p = 0.004), while LDH showed multiple significant positive associations with RET (p = 0.01), TBR (p < 0.001), GGO (p = 0.049) and MOS (p = 0.027). In BAL fluid, lymphocyte fraction had a significant interaction with GGO (p = 0.017). Conclusions: Biomarkers from peripheral blood and BAL may have the potential to differentiate predominantly noninflammatory or fibrotic from active inflammatory radiological ILD patterns. The German Severe Asthma Registry: obesity is associated with asthma parameters abstracts (SGRQ-C) was used. Furthermore, we asked, independent from each other, the patient as well as the treating physician to estimate the global health status of the patient (excellent, good, fair, poor, very poor). Inclusion criteria were a physician's diagnosis of COPD and age ≥ 40 years. Subjects with a history of lung surgery, lung cancer or COPD exacerbation within the last four weeks were excluded. Results: 67 pulmonologists and 6 general practitioners participated and enrolled 1,175 COPD patients. Of those 248 patients did not fulfill the GOLD criteria for COPD (FEV1/ FVC ≥ 0.7) and 77 were excluded due to missing data. Finally, 850 patients (62.8 % men; mean age 66.2 ± 0.3 (SE) years; mean FEV1%pred. 51.5 ± 0.6 (SE)) were analyzed. In 48.7 % of study participants, patients and physicians disagreed on the global health status. In 29.7 % it was estimated better by the physician than by the patient (overrated patients), and in 19.0 % it was underrated. Multivariate regression analysis indicated that overrated patients had a statistically significant better lung function (FEV1), less exacerbations and a lower total SGRQ-C score compared to underrated patients. Conclusions: In stable COPD outpatients, treated by pulmonologists and general practitioners, the global health status, most likely indicating the burden of COPD, tends to be overestimated by physicians in patients with milder airway obstruction and less exacerbations and underestimated in patients with more severe airway obstruction and frequent exacerbations. This discordant perception of global health might severely affect treatment options. Lowering of mean pulmonary artery pressure is a prognostic marker in pulmonary hypertension Background: Treprostinil (TRE), a prostacyclin analog, is effective for the treatment of pulmonary arterial hypertension and non-operable chronic thromboembolic pulmonary hypertension (CTEPH). We hypothesized that a greater change of hemodynamics is of prognostic value. Therefore, we evaluated effects of first-line subcutaneous (sc) TRE in patients with severe pulmonary hypertension (PH) and analyzed the prognostic value of hemodynamic response at 1 year on treatment. Methods: Data was prospectively collected from patients with pre-capillary PH in WHO functional class III or IV, mean right atrial pressure of 10 mm Hg, and/or cardiac index ≤ 2.2 liters/min/m 2 . Patients received first-line scTRE. Dose adjustments were performed individually according to clinical symptoms and side effects. Results: Between 1999 and 2019 138 patients were treated with first-line scTRE. All patients were classified as non-lowrisk at baseline (6MWD > 440, WHO functional class I or II, right atrial pressure < 8 mm Hg and cardiac index ≥ 2.5 L/min/m 2 ). 18 (13 %) patients underwent double lung transplantation, and 59 (42.8 %) died of any cause. Overall survival rates at 1, 5, 10, and 15 years were 91 %, 57 %, 31 % and 29 %. The strongest predictor of outcome was change in mPAP after one year of scTRE. Change in mPAP -18.4 ± 7.9 mm Hg (p = 0.012) was associated with the best subsequent survival of 12.7 ± 1.5 years. Obesity is a risk factor for asthma severity. This study aims to evaluate associations of obesity with severe asthma parameters in the German Severe Asthma Registry including 1065 patients (49 ± 17 yrs, 58 % female, BMI 27 ± 6 kg/m², 26 % obese (BMI ≥ 30 kg/m²)), 107 treated with anti-IgE-and 237 with anti-IL5(R) antibodies. The same proportion of obese patients received biologics as non-obese patients, but obese patients were more frequently on LAMA therapy (60 % vs. 51 % p = 0.01), had more exacerbations (4.5 ± 4/y vs. 3.6 ± 3/y, p = 0.003), worse quality of life and more often uncontrolled asthma (88 % vs. 78 %, p < 0.001), as reflected by worse ACQ (3.2 ± 1.4 vs. 2.8 ± 1.5), ACT (12 ± 5 vs. 15 ± 5) and mAQLQ scores (3.5 ± 1.2 vs. 4.0 ± 1.3; all p < 0.001). Obesity was associated with less blood eosinophils (37 % vs. 53 %, p < 0.001), more neutrophils (6.2 ± 2.8G/L vs. 5.7 ± 2.8G/L, p = 0.02) and lower lung function parameters: FEV1 (1.8 ± 0.7 L vs. 2 ± 0.8 L, p = 0.01) and FVC (2.8 ± 1 L vs. 3.1 ± 1 L, p < 0.001). Non-obese patients were more often non-smokers (42 % in non-obese vs. 27 % in obese, p < 0.001). Conclusions: In our severe asthma cohort, obesity represents a specific phenotype of severe asthma that is significantly associated with exacerbations, worse quality of life, lower blood eosinophil numbers, as well as lower FEV1, and FVC. Discordant perception of global health between COPD outpatients and their physicians -Real world data from the CLARA project of pulmonary exercise hemodynamics with all-cause mortality in patients with normal or mildly elevated pulmonary arterial pressure (PAP) at rest. Methods: Patients with unexplained dyspnea and/or suspected PVD undergoing exercise right heart catheter (RHC) at our PH-clinic were retrospectively analysed. Exercise RHC was performed in case of a resting mPAP < 25 mm Hg. In a first step, dichotomized resting-, submaximal-and maximal exercise hemodynamic variables were analysed using multivariate Cox regression, adjusted for sex and age, to identify prognostic cut-offs. Best cut-off for each variable was defined as the cut-off score with the smallest p-value. In a second step, the relevance of cut-offs, derived from the first model, was assessed using a multivariate model also accounting for age, sex, cardiopulmonary comorbidities, smoking history, and pulmonary resting hemodynamics. Results: 207 patients were included (69 % female, age: 62 ± 13 yr, mPAP: 18 ± 4 mm Hg). Median observational-time was 4.3 yr (IQR: 2.0-8.5) with N = 40 (19 %) mortality events. MPAP/ cardiac ouput (CO)-slope, transpulmonary gradient (TPG)/COslope and pulmonary arterial wedge pressure (PAWP)/CO-slope turned out as sex and age independent predictors of mortality. Best cut-offs were found at 7.5 mm Hg/L/min (mPAP/COslope), 3.9 mm Hg/L/min (TPG/CO-slope) and 6.0 mm Hg/L/ min (PAWP/CO-slope). In the second model, correcting for age, sex, cardiopulmonary comorbidities, smoking history and pulmonary resting hemodynamics, mPAP/CO-slope (HR: 2.84, 95 %CI: 1.22-6.59; p = 0.015), TPG/CO-slope (HR: 2.60, 95 %CI: 1.17-5.82; p = 0.020) and PAWP/CO-slope (HR: 4.92, 95 %CI: 1.95-12.44; p = 0.001) remained significant predictors of allcause mortality. Conclusions: In patients with normal or mildly elevated PAP at rest, pulmonary pressure/CO-slopes are predictors of allcause mortality, independent from age, sex, cardiopulmonary comorbidities and resting pulmonary hemodynamics. Of the annual average of 197 500 insured patients 6.2 to 9.6 % claimed an AO medication. Distribution of the age groups 0-6, 7-17, 18-56 and > 56 years was 4.0, 12.1, 39.4 and 44.6 %, respectively. Based on diagnoses (hospital and sick leave data), age (< 56 years) and drug patterns an asthma cohort was selected (48 % of patients with AO). Annual relative prevalence of selected drug groups is presented in Fig. 1 . From 2013 to 2014 the reduction in reliever drugs is associated with an increase in controller medication, in particular, combinations of inhaled corticosteroids (ICS) and Formoterol (F). Subgroup analyses show that this pattern is consistent in differently defined asthma cohorts, not present in a COPD cohort and more marked in asthma patients seen by a respiratory specialist. 2013 two large studies on the single inhaler treatment (SIT) concept were presented and in 2014 SIT was introduced in the GINA report. Conclusions: Change in asthma treatment recommendation was effectively translated into practice in Burgenland. Pulmonary pressure/flow slopes during exercise as independent predictors of mortality in patients at risk for pulmonary hypertension Philipp Douschan* 1,2 , Vasile Foris 1,2 , Alexander Avian 3 , Teresa Sassmann 1,2 , Horst Olschewski 1,2 , Gabor Kovacs 1,2 Background: Patients with early pulmonary vascular disease (PVD) typically show an abnormal hemodynamic response to exercise. However, it is unknown whether pulmonary exercise hemodynamics are of prognostic relevance in patients with early PVD, independent from pulmonary resting hemodynamics. The aim of this study was to assess the association Fig. 1 | P24 abstracts of extrafine beclomethasone-dipropionate, formoterol-fumarate and glycopyrronium (BDP/FF/ G, Trimbow® 87/5/9 µg) in patients with COPD. Methods: A prospective, multicenter NIS was conducted over 52 weeks in pulmonary and general practices in Austria in 2018/19. Eligible patients with COPD had an indication for treatment with BDP/FF/G according to the summary of product characteristics. In addition to tolerability, lung function, exacerbation rate, symptom scores and COPD assessment Test (CAT) were recorded. Results: 265 patients (male 66 %, mean age 67 years) with moderate to very severe airflow limitation (GOLD Grade 2-4: 96.2 %) and persistent symptoms (GOLD B: 62.3 %, GOLD D: 34 %) according to the 2018 GOLD Report were included. By end of 52 weeks, lung function parameters (FEV1, FEV1 %, and FVC; p < 0.001) and symptoms (cough, sputum and shortness of breath; p < 0.001) improved significantly compared to baseline. A clinically-relevant improvement from baseline in CAT score was observed at week 12 and persisted at week 52 in GOLD B (from 22.1 to 15.3 points; p < 0.001) and GOLD D (from 25.5 to 16.6 points; p < 0.001) patients. A significant reduction of moderate and severe exacerbations over the study period was also observed (57.4 % and 27.3 % respectively; p < 0.001). By end of 52 weeks, 93.7 % continued on the treatment. There were 21 adverse reactions reported, of which 16 were non-serious (e. g. oral mycosis) and five were serious, but none of which were deemed drug-related. Conclusions: Results of this study support the tolerability and effectiveness of BDP/FF/G in patients with COPD in a realworld setting. Patients treated with extrafine BDP/FF/G experienced an improvement in lung function, symptom control and reduction in exacerbations. Tests for diagnostics of COVID-19 -principles and approvals of commercially available tests Rüdiger Siekmeier* 1 , Tanja Grammer 2 , Winfried März 2,3,4 In December 2019 an unknown viral infection was firstly described in a local fish and wild animal market in Wuhan/ China which was identified as a novel coronavirus infection by the Chinese Center for Disease Control and prevention (CCDC) on Jan. 7th 2020 and announced as 2019-new coronavirus disease (2019-nCoV, now COVID-19) by the World Health Organization (WHO) on Feb. 11th 2020. Rapidly spreading across the globe up to begin of August 2020 at least 18 million of infections and 650000 deaths were reported worldwide. Therefore, there was an urgent need of laboratory tests. In our analysis we looked for commercially available COVID-19 tests. At July 31th 2020 at least 280 commercially available tests were described (https:// www.360dx.com/coronavirus-test-tracker-launched-covid-19-tests). Of these, 193 are based on PCR methods (mostly PCR, qPCR) (with Federal Drug Agency (FDA; very most) or Center of Disease Control (CDC; few) Emergency Use Authorization Background: Patients with acute exacerbations of COPD do not only suffer from physical symptoms but also from psychological distress and stress. As pharmacological interventions showed only limited effectiveness in targeting the latter, a need for alternative treatment options emerges. In other chronic conditions, mindfulness interventions are effective in reducing psychological distress and stress. However, research on mindfulness interventions in COPD is still scarce and not focusing on exacerbations. Therefore, the present study reviewed the existing literature and explored the acceptability, feasibility, and implementation of mindfulness interventions focusing on exacerbations in COPD patients. Methods: Firstly, literature examining mindfulness interventions in COPD patients was reviewed. Secondly, a qualitative and explorative study using semi-structured interviews was conducted. The sample consisted of 10 COPD patients (60 % women; M = 74.40 years, SD = 8.30) hospitalised after an acute exacerbation. Data were analysed using thematic analysis. Results: The literature review yielded eight studies, providing preliminary evidence for the feasibility and effectiveness of mindfulness interventions in COPD patients. The qualitative analysis revealed five main findings: (1) Patients express an openness and need for new treatment approaches. (2) Mindfulness is difficult to differentiate from other mind-body concepts. (3) Implementation conditions are crucial for patient's interest. (4) Limitations of the application of interventions must be considered. (5) Not interested patients differ from interested ones. Conclusions: Hospitalized COPD patients showed a strong interest in new treatment approaches like mindfulness interventions. Focusing on mindfulness interventions during exacerbations seem acceptable and feasible. Future studies investigating those are needed and should consider implementation conditions, patients' needs and physical limitations. Background: Systemic Sclerosis is a chronic autoimmune disease characterized by inflammation and tissue remodelling. Increases in the expression and of the AP-1 transcription factor Fra-2 has been shown in the skin of these patients. In mice ectopic overexpression of Fra-2 leads to a systemic sclerosis phenotype, strongly affecting the skin and lung. Fra-2 transgenic mice show pronounced pulmonary inflammation, vascular remodelling and lung fibrosis. Although, the role of several immune cells such as macrophages, B and T lymphocytes has been investigated, the contribution of innate lymphoid cells (ILC) to disease pathogenesis remains elusive. The focus of this study was to determine the development and function of ILC and their role in Scleroderma. Methods: Multi-colour flow cytometry was used to evaluate the inflammatory cell landscape in Fra-2 transgenic mice in a time dependent manner. Primary cells were isolated and functional assays e. g. apoptosis (caspase activation) and proliferation (ki67 staining and cell counts) were performed in vitro. Results: Pronounced changes in the inflammatory profile of the lung were observed in a time dependent manner, with increased numbers of T cells and eosinophils and reduced ILC. Similar changes were also reflected in the blood, spleen and liver. Isolated ILC exhibited decreased proliferation and functional activity. Importantly, reduced numbers and function of ILC was already observed before the first signs of lung fibrosis, as assessed by collagen deposition and lung function measurements. Conclusions: This early dysregulation suggests that ILC play an important role in the development of lung fibrosis in Scleroderma and restoration of ILC could prevent the progression of the disease. (EUA)/with CE-mark/with EUA and CE-mark: 161(12 pending)/60/28) serving as gold standard for virus diagnostics after sampling of nasal/throat swabs in acute infection or other molecular methods (isothermal amplification (4/0/0), CRISPR (2/0/0), sequencing (2/0/0) and others (1/0/0). 3 more tests (1/1/1) are based on immunological antigen detection of virus peptides after sampling of nasal/throat swabs in acute infection which are typically POCT tests based e. g. on immunofluorescence-based lateral flow technology or chromatographic digital immunoassay providing results in a few minutes. 71 tests (50(16 pending, 1 revoked)/34/14) allow measurement of immunoglobulines IgM, IgA and IgG alone/in combination in blood samples and provide information on the immune status after convalescence. Analytical principles of these are different and some (e. g. lateral flow assays) serve for rapid diagnostics. In summary, number and quality of tests rapidly increased. Recent development is based on regulatory guidelines (e. g. https://www.gov.uk/government/publications/how-testsand-testing-kits-for-coronavirus-covid-19-work) and includes also combined tests for discrimination against other diseases (e. g. influenza). Serum tumor maker dynamics as predictive biomarkers in NSCLC chemo-immunotherapy and mono-immunotherapy maintenance -a retrospective cohort study Department of Pulmonology, Johannes Kepler University Linz, Linz, Austria Objectives: To evaluate serum tumor markers (STM) as biomarkers for treatment, monitoring and prognosis in advanced non-small cell lung cancer (NSCLC) treated with chemoimmunotherapy. Methods: Patients having received platinum-based doublet chemotherapy (CHT) and PD-1/PD-L1-directed immune checkpoint inhibitor (ICI) combination therapy were retrospectively followed. Carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9), cytokeratin-19 fragments (CYFRA 21-1) and neuron specific enolase (NSE) were routinely measured at NSCLC diagnosis. The marker with the highest relative elevation was defined "leading STM", its change was assessed between CHT-ICI initiation as well as first mono-ICI maintenance therapy and the respective subsequent restaging. Corresponding computed tomography (CT) evaluations were analyzed according to response evaluation criteria in solid tumors (RECIST). For both CHT-ICI and ICI-maintenance phase, STM and RECIST response were evaluated regarding progression-free (PFS) and overall survival (OS) in Kaplan-Meier analyses. Results: Among 80 CHT-ICI patients (41 % women, mean age 63 years), median PFS was 5 months (M; 4,9) and median OS was 15 M (10,/). PFS was significantly (p = 0.042) longer, when STM concomitantly decreased (9 M (5,12; n = 41)) vs. 5 M (3,6; n = 16). In the 54 (67.5 %) patients who received mono-ICI maintenance, STM decrease was associated with significantly (p < 0.001) longer PFS (16 M (7,/; n = 16) vs. 3.5 M (2,6; n = 22)). Median OS was not reached in most subgroups in both treatment phases. Patients with radiologically stable or progressive disease and concomitant STM decrease vs. increase had similar PFS in the CHT-ICI setting (4 M (3,7; n = 16) vs. 4.5 (2,6; n = 14)), but longer PFS in the mono-ICI maintenance setting (13 M (7,16; n = 10) vs. 3 M (2,4; n = 17)). Employing a retrospective approach, we collected and analyzed data of all patients with advanced non-small-cell lung cancer who received ICI monotherapy with atezolizumab, nivolumab or pembrolizumab at the Kepler university hospital Linz between May 2015 and December 2019. Kaplan-Meier analyses were used to evaluate PFS and OS. Uni-and multivariate Cox-regression analyses were calculated to show the impact of influencing variables. Results: Of 228 patients, 166 persons died (72.8 %). Regarding to the 136 male patients, 99 died (72.8 %). For female patients it was 69 out of 92 (72.8 %). Kaplan-Meier analyses showed no significant difference for PFS (median length 3,5 months, p = 0.273) or OS (median length 10 months, p = 0.592) between men and women. With regards to gender related predictors of outcome like PD-L1 expression or ECOG-Score, we observed considerable differences: PD-L1 expression could be shown a significant predictor for PFS and ECOG status predicted OS in men. However, we could not verify any significant predictors for female patients. Conclusions: In our retrospective research covering 228 participants, we could not verify the higher chance of survival among male patients, frequently mentioned in previous studies. The finding that we could not verify any significant predictors for female patients shows the necessity for further research in that field especially in women. Background: Endothelial cells (EC) represent a key cell type in the homeostatic regulation of vascular and lung function, including vasoreactivity, coagulation, immune processes and barrier function. Disturbances in EC function have been associated with development and progression of pulmonary hypertension, both in its idiopathic form or associated with interstitial lung disease. However, it is not clear whether these functional changes are associated with altered EC composition. Methods: We performed single cell RNA sequencing on pulmonary arteries isolated from donors and pulmonary hypertension patients. Bioinformatics analysis was conducted to gain unbiased insight into EC heterogeneity at the single cell level. Multiplex immunofluorescence staining was combined with confocal imaging of lung tissue samples to assess the spatial heterogeneity of pulmonary artery EC. Results: Our data revealed that EC in adult human pulmonary arteries are composed of three major populations. Each population was characterized by enrichment in a specific set of biological processes determining their distinct functional roles. Background: Systemic sclerosis (SSc) is an autoimmune disorder leading to fibrosis of skin and other internal organs. One SSc hallmark is severe vasculopathy with impaired vascular permeability and tone as an early disease manifestation. Pulmonary complications are the main cause of mortality in patients, but treatment options are still limited. Pirfenidone is approved for the treatment of idiopathic pulmonary fibrosis (IPF), however its effectiveness in SSc-PF is still unknown. Here, we investigated the effectiveness of pirfenidone in a preclinical SSc-PF model, the Fra-2 transgenic (TG) mouse. Methods: Fra-2 TG and wild-type (WT) control mice received either standard or pirfenidone supplemented diet. Pulmonary function testing, fibrosis quantification, inflammatory cell profiling of bronchoalveolar lavage (BAL) and lung tissue as well as transcriptome analysis of lung homogenates was performed. Using in vitro electric cell impedance sensing measurements pirfenidone effects on endothelial cell permeability were analysed. Results: Compared to WT mice, TG mice had decreased lung function and elevated levels of inflammatory cells in BAL and lung. Pirfenidone exacerbated this phenotype further by increasing collagen deposition and worsening lung function. These functional and structural changes were associated with significantly higher lung tissue and BAL inflammation, characterized by predominant eosinophilic infiltration in pirfenidone-treated TG mice. Of note, pirfenidone did not alter lung function, collagen deposition or inflammation in WT mice. Transcriptomic profiling indicated the activation of inflammatory cell recruitment and extravasation pathways with significant downregulation of the endothelial cell barrier protein VE-Cadherin. Further, pirfenidone led to decreased resistance of pulmonary microvascular endothelial cell monolayers in vitro. Conclusions: Pirfenidone was associated with significant deterioration of lung function and elevated inflammatory infiltrates in the lungs of SSc mice. Our study shows that this effect might be due to disturbances of endothelial cell integrity upon pirfenidone treatment especially in diseases with a predisposed vasculature such as SSc. Background: There is a growing interest in metabolic profiling of pulmonary arterial hypertension (PAH) due to current findings suggesting significant metabolic changes causing pulmonary arterial remodeling and linking PAH to insulin resistance. Such findings may have major impact on future diagnostic and therapeutic strategies for PAH. However, most of the studies have enrolled patients with severe disease whose reduced physical activity may have a profound effect on insulin sensitivity. We aimed to directly measure insulin sensitivity in IPAH patients by applying the gold standard method Botnia-clamp. Methods: We assessed insulin sensitivity in five non-diabetic, normal weight patients with severe idiopathic PAH and preserved physical activity in comparison to their age-, sex-, and body composition matched non-diabetic healthy controls. For assessing insulin sensitivity, the hyperinsulinemic-euglycemic (Botnia) clamp was performed in a simultaneous pairwise matched-control manner. Results: In this study we detected no indication of insulin resistance in patients characterized by manifest IPAH but no major limitations in their daily physical activity. Both IPAH and control groups displayed normal efficacy of glycemic control. The Botnia clamp measurements showed no differences in insulin response or insulin sensitivity in any of the IPAH patients when compared to their healthy controls and also the comparison of the groups showed no significant differences. In IPAH, the whole-body glucose disposal capacity in response to insulin infusion showed the same characteristics as in healthy controls. Conclusions: This study does not support insulin resistance to be a primary cause of pulmonary vascular remodeling in IPAH. Multiplex imaging analysis confirmed in situ relative frequencies of EC populations and revealed their characteristic spatial distribution throughout the pulmonary artery tree. Arteries in pulmonary hypertension patients displayed altered composition of EC population characterized by the diminished presence of one cell cluster. Conclusions: In this study, we have revealed EC heterogeneity in the human pulmonary arteries at a single cell resolution and uncovered evidence for their distinct functional specification. Cellular therapy aimed at restoration of the affected cluster or expression of their key genes could serve as a potential therapeutic option in treatment of pulmonary hypertension. Clinical relevance of exercise hemodynamics and right ventricular function in COPD patients asthma may have consulted the internet for self-medication advice. Methods: On July 01 2020, we queried Google Trends for the terms "coronavirus asthma", "-COPD", "-hypertension", "-diabetes" and "-cancer", all representing pre-existing conditions constituting a major risk for Covid-19. When further exploring the health-seeking behavior of patients affected by asthma and/ or COPD during the Covid-19 outbreak, we focused on those therapeutic approaches with the highest RSV world-wide and thus comparable. Results: We observed highest RSV for "coronavirus asthma" followed by "coronavirus diabetes" and "coronavirus cancer", "coronavirus hypertension" ranked fourth together with "coronavirus COPD". Paralleling the world-wide Covid-19 outbreak, highest RSV was seen for the topics "Salbutamol", "Montelukast", "Ipratropium bromide", "Beclometasone", and "Flucticasone propionate", encompassing mainly relievers, followed by inhaled corticosteroids (ICS). Conclusions: Despite other risk factors like hypertension having been largely debated in the media, our analysis revealed highest search volumes for asthma. Considering the GINA guidelines in which the authors explicitly state that asthma treatment should no longer be based solely on short-acting bronchodilators, our data clearly indicates a fail in reaching asthma patients with respective fundamental changes in therapy. Even more alarming is the high search volume for Montelukast, since the FDA released a boxed warning for Montelukast in March 2020 because of serious neuropsychiatric side-effects. Our findings emphasize the urgent need of spreading guidelines and respective updates in a timely manner more intensively in order to reach the general public-especially in a world with an ongoing, potentially life-threatening pandemic. Is there a difference in local disease control between a VATS and thoracotomy approach? Most studies suggest a similar outcome, but nodal upstaging as a quality parameter is frequently reported to be higher in thoracotomy patients. If more positive lymph nodes are missed by VATS, pN0 in these patients should result in a higher failure rate of local disease control. In this study we analyze the difference of VATS to open thoracotomy regarding above mentioned parameters. Methods: The institutional database was queried. Exclusion criteria were pathologic nodal positive status, metastatic disease, tumour size >4 cm, adjuvant/neoadjuvant therapy. 422 patients were included. The VATS cohort included 350 patients, the thoracotomy cohort 72 patients. Results: A VATS approach in patients with pathologic N0 disease did not show a significantly higher rate for local or lymph node recurrence compared to thoracotomy (12.9 % vs. 19.4 %; p = 0.142). There was no difference in disease-free and overall survival comparing the two groups. Comparing the location of recurrence, thoracotomy patients showed a significantly higher rate of metastatic disease (3.04 % vs. 11.54 %; p = 0.014), Beneficial effects of multidisciplinary rehabilitation in post-acute COVID-19 tionnaire related to COVID-19 specific symptoms was filled out by all participants. Results: 12,419 subjects participated (5,984 LEAD participants, 6,440 household members). The projected number of cases according to age and sex for Vienna is 21,504 cases (1.13 %). The cumulative number of positive tested cases in Vienna until May 20th was 3,020. Hence, the projected number is 5.5 to 9.1 times larger than the observed cases. The relative risk of seropositivity by age was highest for children aged 6-9 years [RR 1.21 (CI 0.37-4.01)] and lowest for subjects 65 years and older [RR 0.47 (CI 0.21-1.03)]. Half of the infected subjects developed no or mild symptoms. In a multivariate analysis (Fig. 1) taste and smell disturbances were most strongly related to SARS-CoV-2-specific antibody positivity. The infection probability within households with one confirmed SARS-CoV-2-specific antibody-positive person was 31 %, about 30 times higher than the general ambulatory infection risk. Conclusions: Prevalence rates in Vienna are low (1.13 %) with the highest seroprevalence in young children and lowest in older (≥65 years) inhabitants. Taste and smell disturbances are very prevalent in COVID-19 infected persons and can guide clinicians in diagnosis-and decision making of COVID-19. Distribution and prognostic significance of gluconeogenic and glycolytic phenotypes in nonsmall cell lung cancer Background: Skin prick test (SPT) is a minimal invasive diagnostic test, identifying Type-I-sensitization, which is associated with symptoms as wheezing, atopic dermatitis and rhinitis. Prevalence data vary between countries from 37.7 %-68.6 %. Data about the prevalence in Austria are scarce. Moreover, associated factors for positive SPT have only been investigated in specific age-(e. g. children or adults only) and subgroups (e.g asthmatics).Therefore, our aim was to investigate the prevalence of positive SPT in a general Austrian population, to define associated factors and compare the prevalence and associated factors between childhood and adulthood. Methods: Data was obtained from the LEAD Study, an observational, population-based cohort. We included 11.283 participants with a valid SPT and analyzed two age groups separately: childhood (6-18 yrs; N = 1439) and adulthood (19-82 yrs, N = 9844). Multivariate regression model was used to identify factors associated with positive SPT including socioeconomic status, allergic and/or respiratory diseases, lung function, body composition, lifestyle habits, smoking exposure, pet exposure, and family history. Results: In our study the overall prevalence of a positive SPT is 37.6 % and is higher in male compared to female in all age pins (Fig. 1) . House dust mite and grasses mix are the most prevalent allergens. Factors positively associated with positive SPT in childhood are doctor's diagnosed allergy or asthma and diagnosed parental allergy; in adulthood are doctor's diagnosed allergy or asthma, diagnosed parental allergy, and high socioeconomic status. Smoking (current, former and secondhand) is ally, glycolytic and gluconeogenic gene expression was inferred from The Cancer Genome Atlas (TCGA) datasets. Results: PCK2 was preferentially expressed in the lung adenocarcinoma subtype, while GLUT1 expression was higher in squamous cell carcinoma. GLUT1 and PCK2 were inversely correlated, GLUT1 showing preferential expression in larger tumors while PCK2 was highest in smaller tumors. However, a mixed phenotype showing the presence of both, glycolytic and gluconeogenic cancer cells was frequent. In lung adenocarcinoma, PCK2 expression was associated with significantly improved overall survival compared to glycolytic or mixed tumors, while the opposite was found for GLUT1. PCK1/2 expression was enhanced in metastases compared to primary tumors. The metabolic tumor microenvironment and the 3-dimensional context play an important role in modulating both pathways, since PCK2 expression preferentially occurred at the tumor margin and hypoxia differentially regulated glycolysis and gluconeogenesis in NSCLC cells in vitro. Conclusions: Glycolysis and gluconeogenesis are activated in NSCLC in a tumor size and oxygenation dependent manner and show a differential correlation with outcome. The frequent co-activation of gluconeogenesis and glycolysis in NSCLC should be considered in potential future therapeutic strategies targeting cancer cell metabolism. abstracts assays showed significantly reduced migration of RGS5-/-neutrophils towards chemokines with preserved intra-cellular calcium signaling. Importantly, the attenuated neutrophil migration was associated with activated RhoA, suggesting RhoA as a predominant negative regulator of neutrophil transmigration. Conclusions: Our findings demonstrate the efficacy of silenced RGS5 for suppressing neutrophilic hyperinflammation in two different animal models. The specific effects of RGS5 loss might provide an option for a novel therapeutic intervention in inflammatory lung diseases with recurrent exacerbations, without compromising infection defense mechanisms. Endothelial dysfunction following enhanced TMEM16A activity in human pulmonary arteries Background: Endothelial dysfunction is one of the hallmarks of different vascular diseases, including pulmonary arterial hypertension (PAH). Ion channelome changes have long been connected to vascular remodelling in PAH, yet only recently the focus shifted towards Ca2+-activated Cl-channels (CaCC). The most prominent member of the CaCC TMEM16A has been shown to contribute to the pathogenesis of idiopathic PAH (IPAH) in pulmonary arterial smooth muscle cells, however its role in the homeostasis of healthy human pulmonary arterial endothelial cells (PAECs) and in the development of endothelial dysfunction remains underrepresented. Methods: Using healthy donor (n = 3) and IPAH (n = 3) lungs, we analysed the expression of TMEM16A in primary human PAECs. IPAH was mimicked with selective adenoviral overexpression encoding TMEM16A tagged with mCherry. TMEM16A activity was investigated by patch clamp. Live cell Ca2+ imaging was applied to detect changes in Ca2+ homeostasis. PAEC proliferation, apoptosis, tube-formation, wound healing assay, NO production and measurements of PAECs metabolic state addressed functional consequences of increased TMEM16A activity. The role of endothelial TMEM16A in the tone of pulmonary arteries ex vivo was investigated by wire myography. Results: Here we report enhanced TMEM16A activity in IPAH PAECs. Upon TMEM16A overexpression in healthy primary human PAECs in vitro and in human pulmonary arteries negatively associated with positive SPT in adulthood, but not in childhood. Conclusions: Our study in an Austrian general population identified that 1. positive SPT is highly prevalent, 2. is more prevalent in male, 3. the main allergens observed are grasses mix and house dust mite, and 4. there is a difference in factors associated with SPT in childhood vs adulthood. Neutrophil recruitment in the acute inflammatory phase of interstitial lung disease is determined by RGS5 Methods: Data was obtained from the Austrian LEAD Study, an observational, population-based cohort study. Adults aged 25-82 years with valid LF and metabolic data, including waist circumference (WC) for central obesity, and DXA Scan for VAT (n = 9.157) were included in this analysis. LF was assessed by spirometry pre-and post-bronchodilation (BD). Abnormal LF was defined as FEV1 pre BD 1 involved anatomical structures) had a trend for impaired OS (HR 0.171, p = 0.060) and significantly worse DFS (HR 0.19, p = 0.003).Conclusions: Radical resection of primary malignant PS and CWS offers good long-term outcome with low complication rate despite extended resections. However, extent of disease and subsequent necessity for extended resection is an unfavorable factor for long-term survival. Background: The ESTS EuroLung scores were established to predict postoperative morbidity and mortality in patients undergoing anatomic lung resections. Since its introduction, the EuroLung scores have been updated once and an easy-touse and free-of-charge smart phone app has been created. So far, the scores have not been validated in other patient cohorts. Herein we aimed to elaborate the accuracy of the various EuroLung scores in our VATS cohort. Methods: The EuroLung scores were calculated for a consecutive cohort of 729 patients scheduled for VATS lobectomy. Postoperative complications, as defined and used by the EuroLung scores, were then analyzed in this prospectively maintained database.Results: Overall, the observed complication rate was 10.7 % in the VATS lobectomy database. The EuroLung1 predicted a mean risk of morbidity of 21.7 % with a weak eta correlation (η) (EuroLung1: η = 0.192; 2016 parsimonious EuroLung1: η = 0.167; 2019 parsimonious EuroLung1: η = 0.174). A better coherence was observed with the parsimonious EuroLung1 (2016; 11.8 %) and the current parsimonious EuroLung1 (2019; 11.5 %). Binary logistic regression analysis of the included parameters showed that extended resections and ppoFEV1% were associated with increased complications in the EuroLung1 scores. 30-day mortality was 0.8 % (predicted mortality according to EuroLung2: 1.4 %, parsimonious EuroLung2: 1.11 %) and was associated with ppoFEV1% for both scores and coronary artery disease for the EuroLung2 score only. The EuroLung2 showed a larger area under the ROC curve than the parsimonious EuroLung2 (0.59 vs. 0.57). Again, only a very weak eta correlation between predicted and observed mortality was found for the EuroLung2 (η = 0.002) and the parsimonious EuroLung2 (2016) (η < 0.001).Conclusions: Even though predicted and observed morbidity/mortality rates were comparable in our cohort the scores were not useful to predict the individual risk in this VATS cohort. Therefore, the scores should not be used to permit or refuse surgical therapy. Initial experience with Intercostal catheter for postoperative pain management in VATS Background: Postoperative pain and its management influences patients' rehabilitation, postoperative complications and quality of life. Despite its impact there are no uniform guidelines. Different centers seem to use various strategies for pain management. In this study we aim to analyze pain management regimens after VATS lobectomy used in Austrian thoracic surgery units with a special interest in opioid usage and strategies to avoid opioids.Methods: A questionnaire was designed to assess the current use of regional anesthesia, post-operative pain medication and characteristics of individual pain management regimens. The questionnaire was sent to all thoracic surgery units in Austria, with nine out of twelve departments returning them.Results: Pain management varied between all centers. All departments use regional anesthesia perioperatively. Four out of 9 centers use epidural analgesia or an intercostal catheter for postoperative regional anesthesia in at least 50 % of patients. Two departments follow an opioid restrictive regimen, 5 depending on Visual Analogue Scale (VAS) and two administer opioids on a fixed schedule. Three out of 9 departments use NSAIDs on a fixed schedule. The most used medication is Metamizole (8 out of 9 centers; 6 on a fixed schedule, two depending on VAS) followed by Piritramid (6 out of 9 centers; none as a fixed prescription). All centers reported that their regimen is standardized (with 8 centers basing it on an in-house standard) and all assessed their patients pain scores on a regular basis.Conclusions: There is no standardized postoperative pain management regimen after anatomic VATS resections. There seems to be a trend towards prolonged postoperative regional anesthesia to reduce opioid consumption in some centers. A further prospective study is in preparation to evaluate the feasibility of opioid-free postoperative pain management and its impact on quality of life. Perioperative mortality and morbidity following pneumonectomy for severe inflammatory disease of the lung Background: Some cases of severe pulmonary inflammation are not amenable to conservative treatment. If pneumonectomy is required in highly septic and instable patients the inherent risk of the procedure increases further. In a retrospective study we analysed these patients comparing them to elective pneumonectomy in patients with malignant disease.Methods: During the last 15 years 163 patients (age: 60.2 +/-11.3 years; males: 124, females 39) underwent pneumonectomy. 41 of these cases had underlying severe inflammatory disease (central necrotizing abscess or pulmonary gangrene with accompanying empyema) whereas 122 had resection for malignant tumours. abstracts Results: The inflammatory group was significantly younger (56.4 +/-13.4 vs. 61.5 +/-9.8 years; p = 0.01) and had a significantly lower BMI (22.8 +/-6 vs. 25.6 +/-4.6; p = 0.003) than the malignant group. There were no differences concerning cigarette or alcohol consumption or COPD, coronary artery disease or peripheral arterial occlusive disease. Both the rate of severe perioperative complications (30.3 % vs. 41.4 %) and of perioperative death (3.28 % vs. 34.1 %) were significantly higher in patients with inflammatory disease (p = 0.000). In spite of the high perioperative mortality rate of pneumonectomy in inflammatory disease, 5-years survival rate (38.5 % vs. 26.8 %) showed no statistically significant difference between the two groups.Conclusions: Though sometimes required as a life-saving procedure in severe inflammation of the lung, pneumonectomy in such conditions carries a high perioperative morbidity and mortality. If the first 6 months after pneumonectomy are survived however, the prognosis of this subgroup is fair. Problem at the pneumonectomy stump. Salvage by myoplastic closure Background: For primary closure when material is lacking and for the treatment of bronchial stump dehiscence following pneumonectomy a variety of methods such as pericardial or omental flap as well as myoplastic techniques have been advocated. We present our experience with myoplasty for closure of the main bronchus stump.Methods: Retrospective analysis of 163 pneumonectomies within the last 15 years (age: 60.2 +/-11.3 years; males: 124, females 39). In 9 patients (5.5 %) problems at the bronchial stump were present (6 on the right and 3 on the left side), thereof 3 primary impossibilities of direct closure and 6 secondary dehiscences.Results: In one case dehiscence occurred one day after pneumonectomy, in the remaining 4 patients dehiscence became evident after a mean of 17.2 days (6-30 days). The 3 impossibilities of direct closure of the stump derived from necrosis and fistula following bifurcational stenting in lung cancer, from lack of viable bronchial tissue during completion pneumonectomy following left-sided sleeve resection and from bronchial necrosis in aspergillosis. Closure of the stump was done by pedicled pectoralis major flap in 6, by pedicled diaphragmatic flap in 2 and by pedicled sternoleidomastoideus flap in one patient. If deemed necessary, second-look procedures and/or thoracostomy and VAC were additionally used. One patient did not survive his septic condition and died within 30 days (11.1 %). The overall 5 years survival rate was 50 %.Conclusions: Pedicled myoplastic flaps provide reliable closure even in detrimental cases of dehiscence of the main bronchus stump.Publisher's Note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.