Interstitial lung disease with autoantibodies against aminoacyl-tRNA synthetases in the absence of clinically apparent myositis

Semin Arthritis Rheum. 1996 Aug;26(1):459-67. doi: 10.1016/s0049-0172(96)80026-6.


Autoantibodies against aminoacyl-tRNA synthetases (antisynthetases) have been found to be highly specific for polymyositis and dermatomyositis and to correlate strongly with complicating interstitial lung disease (ILD). We describe the clinical presentations and course of 10 patients with ILD and anti-synthetase antibodies in whom underlying myositis was not clinically evident. Anti-PL-12 antibodies (antialanyl-tRNA synthetase) were most common (60%), followed by anti-Jo-1 (antihistidyl-tRNA synthetase) and anti-OJ (anti-isoleucyl-tRNA synthetase) (20% each). All 10 patients had anticytoplasmic antibodies by indirect immunofluorescence on HEp-2 cells. Five of 10 presented with features of connective tissue disease, whereas two presented with acute respiratory failure, two with insidious onset of diminished exercise tolerance, and one with persistent cough. All but one patient received corticosteroids, four were given oral cyclophosphamide, and two azathioprine. ILD resolved or stabilized in five patients (50%), and progressed in four (40%). The "antisynthetase syndrome" may occur in the absence of clinical myositis, and the ILD in these patients is usually responsive to therapy. Antisynthetase testing should be considered in patients with ILD who have a cytoplasmic pattern by antinuclear antibody (ANA) testing on HEp-2 cells, because early recognition and treatment of such patients affects their clinical course.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adult
  • Amino Acyl-tRNA Synthetases / immunology*
  • Amino Acyl-tRNA Synthetases / metabolism
  • Autoantibodies / blood*
  • Biopsy
  • Cells, Cultured / immunology
  • Connective Tissue Diseases / enzymology
  • Connective Tissue Diseases / immunology
  • Disease Progression
  • Female
  • Fluorescent Antibody Technique
  • Follow-Up Studies
  • Humans
  • Liver / cytology
  • Lung Diseases, Interstitial / enzymology
  • Lung Diseases, Interstitial / immunology*
  • Lung Diseases, Interstitial / mortality
  • Male
  • Middle Aged
  • Polymyositis / enzymology
  • Polymyositis / immunology
  • Pulmonary Fibrosis / enzymology
  • Pulmonary Fibrosis / immunology


  • Autoantibodies
  • Amino Acyl-tRNA Synthetases