Interstitial lung disease in antisynthetase syndrome without clinical myositis

Akioka

et al. 2020

Modern Rheumatology

Juvenile dermatomyositis is the most common type of juvenile idiopathic inflammatory myopathy mainly affecting the skin and proximal muscles. We have published the Japanese version of 'Clinical practice guidance for juvenile dermatomyositis (JDM) 2018 'consisting of a review of articles in the field and evidence-informed consensus-based experts' opinion on the treatment strategy in collaboration with The Pediatric Rheumatology Association of Japan and The Japan College of Rheumatology under the financial support by 'Research on rare and intractable diseases, Health and Labor Sciences Research Grants'. This article is a digest version of the Japanese guidance.

Section: Interstitial Lung Diseasesmentioning

confidence: 99%

Clinical practice guidance for juvenile dermatomyositis (JDM) 2018-Update

Akioka

et al. 2020

Modern Rheumatology

Clin Med Insights Circ Respir Pulm Med

“…Several case series revealed the potential efficacy of MMF in the stabilization of progressive ILD and the reduction of corticosteroid dose in ILD with connective tissue disease (CTD) patients, including PM/DM. 42,51-58 An open trial of MMF was conducted in 28 patients with ILD in CTD, including five patients with PM/DM. These patients received MMF (30 mg/kg/day), although pulmonary function tests (FVC and diffusion lung capacity for carbon monoxide [DLco]) showed no significant improvement at 18-month follow-up.…”

Section: Conventional Agentsmentioning

confidence: 99%

Recent Treatment of Interstitial Lung Disease with Idiopathic Inflammatory Myopathies

Kawasumi

Gono

Kawaguchi

et al. 2015

Interstitial lung disease (ILD) is a prognostic factor for poor outcome in polymyositis (PM)/dermatomyositis (DM). The appropriate management of ILD is very important to improve the prognosis of patients with PM/DM. ILD activity and severity depend on the disease subtype. Therefore, clinicians should determine therapeutic strategies according to the disease subtype in each patient with PM/DM. Anti–melanoma differentiation-associated gene 5 antibody and hyperferritinemia predict the development and severity of rapidly progressive (RP) ILD, particularly in East Asian patients. Combination therapy with corticosteroids, intravenous cyclophosphamide pulse, and calcineurin inhibitors should be administered in RP-ILD. In contrast, patients with anti–aminoacyl-tRNA synthetase (ARS) show better responses to corticosteroids alone. However, ILDs with anti-ARS often display disease recurrence or become refractory to corticosteroid monotherapy. Recent studies have demonstrated that the administration of tacrolimus or rituximab in addition to corticosteroids may be considered in ILD patients with anti-ARS. Large-scale, multicenter randomized clinical trials should be conducted in the future to confirm that the aforementioned agents exhibit efficacy in ILD patients with PM/DM. The pathophysiology of ILD with PM/DM should also be elucidated in greater detail to develop effective therapeutic strategies for patients with ILD in PM/DM.

Respiratory Medicine Case Reports

“…Total 30 cases reported, 17 male patients and 13 female patients. Only 43% of the cases presented with lung symptoms alone [2,4,7,10,[16], [17], [18],[23], [24], [25],29,32], while 57% of the cases presented with lung and muscle symptoms simultaneously [1,5,8,[11], [12], [13], [14], [15],[19], [20], [21], [22],[26], [27], [28],30,31]. We did not review any cases that presented with muscle symptoms alone.…”

Section: Resultsmentioning

confidence: 99%

Acute hypoxic respiratory failure secondary to antisynthetase syndrome: A case report and review of literature

Cancel

Song

2019

Objective Antisynthetase syndrome is a condition that includes interstitial lung disease and inflammatory myositis in its definition. The interstitial lung disease of this syndrome can vary in severity and if not identified soon enough, can lead to severe respiratory failure. Here we present a patient who had a working diagnosis of acute eosinophilic pneumonia. He initially improved after prolonged hospitalization and course of high dose steroids. CT chest revealed interval improvement in his bilateral ground glass and reticular opacities but residual fibrotic interstitial lung disease. However, he decompensated subsequently with relapsed hypoxia during activity. We hope that this review will bring awareness to antisynthetase syndrome and provide tools for earlier diagnosis and treatment. The primary objective of this study was to review presenting symptoms, diagnosis, treatment and outcomes. This review is unique because we focused on antisynthetase syndrome that initially manifested with lung symptoms rather than myositis or skin changes. Methods We have performed a comprehensive review of 30 cases of antisynthetase syndrome in the literature (including our case). Results Total 30 cases reported, 17 male patients and 13 female patients. Only 43% of the cases presented with lung symptoms alone, while 57% of the cases presented with lung and muscle symptoms simultaneously. Conclusion This supports the fact that antisynthetase syndrome most commonly presents with lung and muscle manifestations simultaneously. The fact that our case presented with lung findings alone led to the delay in his diagnosis.