A Review of MDA-5 Dermatomyositis and Associated Interstitial Lung Disease

Author:

Bhandari Sambhawana1ORCID,Zickuhr Lisa1ORCID,Baral Maun Ranjan2,Bhalla Sanjeev3,Jones Heather4,Bucelli Robert5,Sen Deepali1

Affiliation:

1. Division of Rheumatology, Department of Medicine, Washington University School of Medicine in St. Louis, St. Louis, MO 63110, USA

2. Division of Hospital Medicine, Department of Medicine, Washington University School of Medicine in St. Louis, St. Louis, MO 63110, USA

3. Department of Radiology, Washington University in St. Louis, St. Louis, MO 63110, USA

4. Meramec Dermatology, St. Louis, MO 63110, USA

5. Department of Neurology, Washington University in St. Louis, St. Louis, MO 63110, USA

Abstract

Anti-melanoma differentiation-associated gene 5 (MDA-5) dermatomyositis (DM) is noteworthy for its association with rapidly progressive interstitial lung disease (RP-ILD), vasculopathy, and distinctive cutaneous features. First identified in a Japanese cohort in 2005, MDA-5 DM carries a significant mortality risk, emphasizing the crucial need for early diagnosis. This review explores the pathogenesis, clinical presentation, diagnosis, management, and prognosis of MDA-5 DM and ILD and includes new research and recommendations regarding disease management.

Publisher

MDPI AG

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