Myositis-related interstitial lung disease and antisynthetase syndrome

J Bras Pneumol. Jan-Feb 2011;37(1):100-9. doi: 10.1590/s1806-37132011000100015.
[Article in English, Portuguese]

Abstract

In patients with myositis, the lung is commonly involved, and the presence of anti-aminoacyl-tRNA synthetase (anti-ARS) antibodies marks the presence or predicts the development of interstitial lung disease (ILD). A distinct clinical entity-antisynthetase syndrome-is characterized by the presence of anti-ARS antibodies, myositis, ILD, fever, arthritis, Raynaud's phenomenon, and mechanic's hands. The most common anti-ARS antibody is anti-Jo-1. More recently described anti-ARS antibodies might confer a phenotype that is distinct from that of anti-Jo-1-positive patients and is characterized by a lower incidence of myositis and a higher incidence of ILD. Among patients with antisynthetase syndrome-related ILD, the response to immunosuppressive medications is generally, but not universally, favorable.

Publication types

  • Review

MeSH terms

  • Amino Acyl-tRNA Synthetases / blood*
  • Antibodies, Antinuclear / blood
  • Antigens, Human Platelet / blood
  • Autoantibodies / blood*
  • Humans
  • Lung Diseases, Interstitial / diagnosis
  • Lung Diseases, Interstitial / immunology*
  • Lung Diseases, Interstitial / therapy
  • Myositis / diagnosis
  • Myositis / immunology*
  • Myositis / therapy

Substances

  • Antibodies, Antinuclear
  • Antigens, Human Platelet
  • Autoantibodies
  • Jo-1 antibody
  • Amino Acyl-tRNA Synthetases

Supplementary concepts

  • Antisynthetase syndrome