title: Pulmonary pathologic manifestations of anti-glycyl-tRNA synthetase (anti-EJ)-related inflammatory myopathy.
abstract: OBJECTIVE
  Antisynthetase syndromes are a subset of the idiopathic inflammatory myopathies characterised by the presence of autoantibodies to aminoacyl transfer-RNA synthetases (ARS) and monotypic clinical features including Raynaud phenomenon, fever, non-erosive inflammatory arthritis and hyperkeratotic skin changes ('mechanic's hands'). Interstitial lung disease (ILD) is particularly common in ARS syndromes, affecting up to 90% of patients.
  
  
  METHODS
  Four patients with ARS syndrome who possessed anti-glycyl-tRNA synthetase (anti-EJ) autoantibodies were retrieved from the University of Pittsburgh database. We report their clinical, radiographic and histopathologic findings.
  
  
  RESULTS
  Patients presented with dyspnoea accompanied by Raynaud phenomenon and 'mechanic's hands'. Lung disease was the first manifestation in all four patients (100%) who were all amyopathic. High-resolution CT of the chest showed patchy opacities and consolidations in two patients (50%) whose surgical lung biopsies revealed organising diffuse alveolar damage (DAD), and lower lung zone predominant reticular infiltrates and traction bronchiectasis without honeycomb change in two patients (50%) whose surgical lung biopsies revealed usual interstitial pneumonia (UIP). Mild lymphoplasmacytic inflammation and few scattered lymphoid aggregates were present, but we found no pathognomonic histopathologic features of anti-EJ ARS syndrome. Serologic testing revealed no other autoantibodies. All patients responded to immunosuppressive therapy.
  
  
  CONCLUSIONS
  Identifying ARS-associated autoantibodies in ILD patients with or without myopathy is desirable because patients may respond well to immunosuppressive therapy, and their prognosis is better than that of patients with idiopathic forms of DAD or UIP.
authors.affiliation: Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Frank
authors.initials: F
authors.lastname: Schneider
authors.affiliation: Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Samuel A
authors.initials: SA
authors.lastname: Yousem
authors.affiliation: Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: David
authors.initials: D
authors.lastname: Bi
authors.affiliation: Division of Pulmonary, Allergy and Critical Care Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Kevin F
authors.initials: KF
authors.lastname: Gibson
authors.affiliation: Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Chester V
authors.initials: CV
authors.lastname: Oddis
authors.affiliation: Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Rohit
authors.initials: R
authors.lastname: Aggarwal
mesh_terms: D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000276:immunology; D005260:Female; D006032:Glycine-tRNA Ligase / Q000276:immunology*; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000000981:diagnostic imaging / Q000276:immunology / Q000473:pathology*; D011859:Radiography
chemical_list: D001323:Autoantibodies; D006032:Glycine-tRNA Ligase
keywords: Autoantibody; Inflammation; Lung; Muscle; fibrosis