Anti-MDA5 dermatomyositis: an update from bench to bedside

Abstract

Purpose of review: This review summarizes the recent developments about anti-MDA5 antibody positive dermatomyositis with a focus on its pathogenesis, clinical features and treatment options of rapidly progressive interstitial lung disease, its most ominous complication.

Recent findings: Anti-MDA5+ dermatomyositis has a heterogeneous clinical spectrum with different patient subsets exhibiting widely different outcomes; severe acute interstitial lung disease is the main factor impacting prognosis. The pathogenetic role of anti-MDA5 antibodies is an active area of investigation.

Summary: Anti-MDA5+ dermatomyositis has a wider spectrum of manifestations than previously thought. A high index of suspicion is needed not to miss atypical presentations. In the setting of acute interstitial lung involvement, once a confident diagnosis is made, an aggressive approach with early combined immunosuppression affords the best chances of survival.

Box 1

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FIGURE 1

Proposed general pathogenetic model of the anti-MDA5 syndrome. DAMP, damage-associated molecular pattern; MDA5, melanoma differentiation antigen 5; PAMP, pathogen-associated molecular pattern. Icons made by Freepik from Flaticon.com.