"Amyopathic" MDA5-positive dermatomyositis with severe lung involvement presenting with net myositic morphological features - insights from an autopsy study

  • Benjamin Englert
  • Carsten Dittmayer
  • Hans-Hilmar Goebel
  • Udo Schneider
  • Marie-Therese Holzer
  • Akinori Uruha
  • Werner Stenzel

Beteiligte Einrichtungen


Anti-MDA5-positive dermatomyositis (MDA5-DM) often presents with extramuscular, especially pulmonary and skin manifestations, and apparent clinical signs of frank myositis can be missing (so called amyopathic DM). We hereby present two male patients who died from respiratory failure during the course of MDA5-DM. While overt signs of myositis or any skin involvement were absent at admission to hospital we noticed conspicuous inflammatory alterations in various skeletal muscles morphologically, showing different degrees of affection. Furthermore, pathological changes of the lungs compatible with rapid progressive interstitial lung disease and characteristic cutaneous vasculoocclusive features were identified at autopsy. This observation shows that muscles and skin are subclinically affected in a widespread fashion, hence subtle signs of muscle involvement should be sought after in anti-MDA5-positive patients with predominant lung affection to ensure adequate treatment.

Bibliografische Daten

StatusVeröffentlicht - 03.2024

Anmerkungen des Dekanats

Copyright © 2024 The Author(s). Published by Elsevier B.V. All rights reserved.

PubMed 38354588