Successful tocilizumab treatment for rapidly progressive interstitial lung disease with anti-MDA5-positive juvenile dermatomyositis: a case report and literature review

Abstract

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BackgroundCurrently, no established integrated treatment regimen exists for anti-melanoma differentiation-associated gene 5 (anti-MDA5)-positive juvenile dermatomyositis (JDM) complicated by rapidly progressive interstitial lung disease (RP-ILD). We present a case of refractory anti-MDA5-positive JDM with RP-ILD that was successfully treated using a combination of tocilizumab and plasma exchange, along with a review of the relevant literature.MethodsA literature review was conducted to gain insights into the clinical features and treatment strategies for managing refractory anti-MDA5-positive JDM complicated by RP-ILD.ResultsWe report a case of successful management of anti-MDA5-positive JDM complicated by RP-ILD using a combination of immunosuppressive agents, plasma exchange, and tocilizumab.ConclusionTocilizumab may serve as an effective adjunctive treatment option for patients with refractory anti-MDA5-positive JDM complicated by RP-ILD who do not respond to conventional intensive immunosuppressive therapies.

Keywords

• anti-MDA5
• juvenile dermatomyositis
• rapidly progressive interstitial lung disease
• tocilizumab
• plasma exchange