key: cord-0941579-5v43yg4p
authors: Takeda, Yukihisa; Ono, Mariko; Kinoshita, Hayato; Nagatomo, Yoko; Miyauchi, Hiroki; Tsukamoto, Hiroshi; Nakamura, Hiroyuki; Aoshiba, Kazutetsu
title: Acute respiratory distress syndrome relapsing in 10 months with an initial manifestation of polymyositis
date: 2021-12-06
journal: Clin Case Rep
DOI: 10.1002/ccr3.5147
sha: 22fb6dbcdf0b7cf4efba12c29d46134bc6e27c04
doc_id: 941579
cord_uid: 5v43yg4p

Autoimmune disorders are an important cause of acute respiratory distress syndrome (ARDS). We report a case of a patient with steroid‐responsive ARDS that relapsed in 10 months with an initial manifestation of seronegative polymyositis. ARDS associated with polymyositis may develop earlier than myopathy and may relapse later.

2019 (before the COVID-19 outbreak). He had no history of cigarette smoking, alcohol consumption, or sick contacts. He had a frequent cough, tachypnea (40 breaths per minute), low-grade fever (37.2°C), and hypoxemia (PaO 2 , 50.2 mm Hg on room air). He did not have wheezes or lung crackles and abnormal heart sounds on auscultation. Edema, skin rash, muscle weakness, myalgia, and arthralgia were absent. Blood tests revealed leukocytosis (10,300 cells/μl with 76% neutrophils, 2.0% eosinophils, and 14.0% lymphocytes) with high C-reactive protein levels (5.27 mg/dl). He had normal liver and renal function tests (aspartate aminotransferase 25 IU/L, normal <38 IU/L; alanine aminotransferase 30 IU/L, normal <40 IU/L; blood urea nitrogen 13.6 mg/dl, normal <20 mg/dl; and creatinine 1.04 mg/dl, normal <1.10 mg/ dl) and no elevation of creatinine kinase (155 IU/L, normal <170 IU/L). Autoimmune screening did not identify any abnormalities, including anticyclic citrullinated peptide, anti-nuclear antibodies, anti-double-stranded DNA antibodies, anti-proteinase 3 (PR3) antibodies, anti-myeloperoxidase (MPO) antibodies, anti-Scl-70 antibodies, anti-Sjögren's syndrome-related antigen A (SSA/ Ro52) antibodies, anti-aminoacyl-transfer RNA synthetase (ARS) antibodies, anti-Jo-1 antibodies, and antimelanoma differentiation-associated gene 5 (MDA5) antibodies. Chest X-ray and computed tomography (CT) scan showed diffuse ground-glass opacification and consolidation in bilateral lung fields ( Figure 1A and B). On the day of admission, the patient's condition deteriorated rapidly and he received noninvasive intermittent positive pressure ventilation (NPPV). The diagnosis of rapidly progressive interstitial lung disease with autoimmune disorders, such as severe inflammatory myopathy-related interstitial lung disease, could not be ruled out. Based on the diagnosis of ARDS of unknown etiology (PaO 2 /FiO 2 235 with a positive end-expiratory pressure of 5 cmH 2 O), high-dose (1000 mg/day) intravenous (IV) methylprednisolone therapy was initiated. Empiric antibiotics (IV piperacillin-tazobactam and levofloxacin) were also given, although blood culture and urinary pneumococcal and Legionella antigen tests were negative. After 3 days of steroid pulse therapy, the patient improved dramatically and was weaned from NPPV and, thereafter, from oxygen support. The dose of IV methylprednisolone was reduced to half every 3 days and later it was switched to oral prednisolone (60 mg/day), which was also gradually reduced. Chest CT scan taken on the 13th day of admission revealed almost complete disappearance of abnormal shadows from the lung field ( Figure 1C) . He was discharged without dyspnea on the 26th day of admission. The steroid was tapered down gradually and discontinued 7 months after discharge. Although the patient had been asymptomatic for a while, he had a relapse of dyspnea in 10 months later after the first onset of ARDS. Upon the second admission, he had hypoxemia (PaO 2 64 mm Hg) on O 2 5L/ min via face mask and started receiving NPPV therapy. Chest CT scan showed a mixture of diffuse ground-glass opacification and consolidation similar to roentgenological patterns observed previously ( Figure 1D ). The findings of physical examination and blood tests were not significantly different from the previous admission except that he had grasping pain in both thighs, proximal muscle weakness in extremities, and elevation of serum creatinine kinase (1741 IU/L). He had no skin eruptions, such as nail-bed telangiectasia, heliotrope rash, Gottron's papules, Raynaud's phenomenon, and hyperkeratotic lesions on his fingers (mechanic's hands). The short-tau inversion recovery sequence (STIR) of magnetic resonance imaging (MRI) showed inflammatory changes in both hamstring muscles ( Figure 1E ). However, the Euroline myositis line blot assay showed negative results for either myositis-specific antibodies (Jo-1, PL-7, PL-12, EJ, SRP, Mi-2, MDA5, and TIF1-γ) or myositis-associated antibodies (Ku, PM-Scl100, Scl-70, and SSA/Ro52). From these findings, the diagnosis of ARDS that relapsed along with an initial manifestation of seronegative PM was made. After 3 days of high-dose (1,000 mg daily) IV methylprednisolone therapy, the patient's dyspnea and muscle Discharge Discharge weakness improved dramatically and NPPV therapy was discontinued. Additionally, the diffuse abnormal shadows on the chest CT scan ( Figure 1F ) and the high signal on STIR MRI of the hamstring muscles ( Figure 1G ) disappeared. The dose of IV methylprednisolone was gradually reduced to 40 mg/day prednisolone, when the patient was discharged without respiratory and muscular symptoms on the 26th day of the second admission ( Figure 2 ). The steroid was tapered down gradually and discontinued 6 months after discharge.

This patient had several unique clinical features. First, he showed a good clinical response to steroid pulse therapy. It is reported that patients with PM/DM developing diffuse alveolar damage, which largely corresponds to the histopathology of ARDS, have a poor prognosis and sometimes fatal consequences despite high-dose steroid therapy. 3, 9 Since the steroid therapy led to the complete resolution of both respiratory and muscular symptoms in this patient, we suspect that ARDS resulted from a systemic inflammatory response, which involved the lungs and muscles, and not from diffuse alveolar damage to the lungs. Second, ARDS preceded the manifestation of myopathy, which became evident when ARDS relapsed 10 months later. ILD in PM/DM can precede or follow myopathy or occur simultaneously with it. 10 However, recurrent and remittent ARDS preceding the diagnosis of DM/PM is rare because of a potentially poor prognosis of ARDS. In this context, patients with anti-ARS antibodies have been shown to respond well to steroid therapy, but with frequent disease recurrence. 3, 11 Third, this patient had no identifiable autoantibodies on the Euroline myositis line blot assay; however, the result does not exclude the diagnosis of PM because of the low prevalence of autoantibodies in IIM, as exemplified by a recent study showing that 38% of the patients with IIM were autoantibody-negative. 12 Further studies might identify new autoantigenic targets in patients with PM/DM who are currently regarded as autoantibody-negative. 6 The differential diagnosis of ARDS should include a pulmonary manifestation of PM/DM, which may precede myopathy symptoms. ARDS without clear etiology could be a manifestation of a latent form of PM/DM despite the seronegative profile.

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Interstitial lung disease in polymyositis and dermatomyositis

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Diffuse alveolar damage in patients with dermatomyositis: a six-case series

Anti-aminoacyl-tRNA synthetase antibodies in clinical course prediction of interstitial lung disease complicated with idiopathic inflammatory myopathies

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The authors declare no relevant acknowledgments.

The authors have no conflicts of interest to declare.

YT and KA wrote the first draft. MO, HK, YN, HM, HT, and HN revised the manuscript.

Written consent from the patient was obtained for submission and publication of the case details and images.

The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions.

Kazutetsu Aoshiba https://orcid. org/0000-0002-9490-9754