A Review of Antisynthetase Syndrome-Associated Interstitial Lung Disease

Int J Mol Sci. 2024 Apr 18;25(8):4453. doi: 10.3390/ijms25084453.

Abstract

Our objective in this review article is to present a clinical case of a patient with antisynthetase syndrome (ASyS) and provide an overview of the pathogenesis, classification criteria, antibody profiles, clinical features, and current knowledge of treatment options, focusing on interstitial lung disease (ILD). ASyS is an uncommon autoimmune disease with a heterogenous clinical presentation characterized by the presence of autoantibodies against an aminoacyl-tRNA synthetase and manifested by myositis, fever, inflammatory arthritis, Raynaud's phenomenon, mechanics hands, and ILD. ASyS-associated ILD (ASyS-ILD) is the most serious complication of ASyS, which may evolve to rapidly progressive ILD; therefore, it often requires thorough clinical and radiologic evaluation including recognition of a specific clinical phenotype associated with the antisynthetase antibodies (ASAbs) to guide therapeutic interventions.

Keywords: IIM; anti-Jo-1 antibody; antisynthetase antibodies; antisynthetase syndrome; idiopathic inflammatory myopathy; interstitial lung disease.

Publication types

  • Review
  • Case Reports

MeSH terms

  • Amino Acyl-tRNA Synthetases / immunology
  • Autoantibodies* / immunology
  • Female
  • Humans
  • Lung Diseases, Interstitial* / complications
  • Lung Diseases, Interstitial* / etiology
  • Lung Diseases, Interstitial* / immunology
  • Middle Aged
  • Myositis* / complications
  • Myositis* / diagnosis
  • Myositis* / immunology

Substances

  • Amino Acyl-tRNA Synthetases
  • Autoantibodies

Supplementary concepts

  • Antisynthetase syndrome

Grants and funding

This research received no external funding.