An Atypical Clinical Course of Anti-MDA5 Antibody-positive Interstitial Lung Disease in a Patient with Three Deteriorations in 9 years

Intern Med. 2017;56(3):341-346. doi: 10.2169/internalmedicine.56.6856. Epub 2017 Feb 1.

Abstract

Anti-MDA5 antibody-positive patients with clinically amyopathic dermatomyositis (CADM) are at high risk of developing rapidly progressive interstitial lung disease (ILD), which is associated with a high mortality rate. Approximately half of the patients with ILD recover; however, the long-term clinical course of these patients has not been fully reported and is not completely understood. This report describes the atypical clinical course of an anti-MDA5 antibody-positive CADM patient who experienced three deteriorations of ILD in 9 years. These findings indicate that the ILD in anti-MDA5 antibody-positive patients may not only be rapidly progressive, but may also be chronic and recurrent.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Anti-Idiotypic / immunology*
  • DEAD-box RNA Helicases / immunology
  • Dermatomyositis / complications
  • Dermatomyositis / immunology*
  • Humans
  • Intercellular Signaling Peptides and Proteins
  • Interferon-Induced Helicase, IFIH1 / immunology
  • Lung Diseases, Interstitial / complications
  • Lung Diseases, Interstitial / diagnosis
  • Lung Diseases, Interstitial / immunology*
  • Male
  • Middle Aged
  • Peptides / immunology*

Substances

  • Antibodies, Anti-Idiotypic
  • CADM-140 peptide, human
  • Intercellular Signaling Peptides and Proteins
  • Peptides
  • DEAD-box RNA Helicases
  • Interferon-Induced Helicase, IFIH1

Supplementary concepts

  • Amyopathic dermatomyositis