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Pulmonary pathologic manifestations of anti-glycyl-tRNA synthetase (anti-EJ)-related inflammatory myopathy
  1. Frank Schneider1,
  2. Samuel A Yousem1,
  3. David Bi1,
  4. Kevin F Gibson2,
  5. Chester V Oddis3,
  6. Rohit Aggarwal3
  1. 1Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA
  2. 2Division of Pulmonary, Allergy and Critical Care Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA
  3. 3Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA
  1. Correspondence to Dr Frank Schneider, Department of Pathology C605, University of Pittsburgh Medical Center, 200 Lothrop St, Pittsburgh, PA 15213, USA; schneiderf{at}upmc.edu

Abstract

Aims 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.

  • Lung
  • Muscle
  • Autoantibody
  • fibrosis
  • Inflammation

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