A case of anti-MDA5-positive rapidly progressive interstitial lung disease in a patient with clinically amyopathic dermatomyositis ameliorated by rituximab, in addition to standard immunosuppressive treatment

Mod Rheumatol. 2017 May;27(3):536-540. doi: 10.3109/14397595.2015.1014140. Epub 2015 Mar 12.

Abstract

Rapidly progressive interstitial lung disease (RP-ILD) in patients with clinically amyopathic dermatomyositis (CADM) associated with antibodies to melanoma differentiation-associated gene5 (MDA5) results in a high mortality rate. We experienced a case of anti-MDA5-positive RP-ILD of CADM which showed a response to rituximab, although there was no significant effect due to standard immunosuppressive treatment. This case suggests that rituximab has the potential to offer an effective agent for the treatment of anti-MDA5-positive RP-ILD of CADM.

Keywords: Anti-MDA5 antibody; Dermatomyositis; Interstitial lung disease; Rituximab.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Dermatomyositis / complications
  • Dermatomyositis / diagnosis*
  • Dermatomyositis / drug therapy
  • Dermatomyositis / immunology
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Interferon-Induced Helicase, IFIH1 / immunology
  • Lung Diseases, Interstitial / complications
  • Lung Diseases, Interstitial / diagnosis*
  • Lung Diseases, Interstitial / drug therapy
  • Rituximab / therapeutic use*

Substances

  • Immunosuppressive Agents
  • Rituximab
  • IFIH1 protein, human
  • Interferon-Induced Helicase, IFIH1

Supplementary concepts

  • Amyopathic dermatomyositis