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.

MeSH

Lung Diseases, Interstitial, Rheumatic Diseases, Immunosuppression Therapy, Dermatomyositis, Arthritis

Cited by

• Dual Autoimmunity: A Case Report of the Sequential Development of Systemic Lupus Erythematosus in a Patient With Anti-MDA5 Dermatomyositis. Cureus, 2025
• Clinical Features of Dermatomyositis Associated with Myositis-Specific Antibodies in Moroccan Patients. Clin Pract, 2025
• Pneumomediastinum in MDA5+ Dermatomyositis: A Case Series. Mediterr J Rheumatol, 2025
• Disease-associated immune cell endotypes in anti-MDA5-positive dermatomyositis using unbiased hierarchical clustering. Front Immunol, 2024
• Interferon and autoantigens: intersection in autoimmunity. Front Med (Lausanne), 2023