Rheumato (Feb 2024)

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

  • Sambhawana Bhandari,
  • Lisa Zickuhr,
  • Maun Ranjan Baral,
  • Sanjeev Bhalla,
  • Heather Jones,
  • Robert Bucelli,
  • Deepali Sen

DOI
https://doi.org/10.3390/rheumato4010004
Journal volume & issue
Vol. 4, no. 1
pp. 33 – 48

Abstract

Read online

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.

Keywords