Abstract | AIMS: 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.
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Authors | Frank Schneider, Samuel A Yousem, David Bi, Kevin F Gibson, Chester V Oddis, Rohit Aggarwal |
Journal | Journal of clinical pathology
(J Clin Pathol)
Vol. 67
Issue 8
Pg. 678-83
(Aug 2014)
ISSN: 1472-4146 [Electronic] England |
PMID | 24891607
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Copyright | Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions. |
Chemical References |
- Autoantibodies
- Glycine-tRNA Ligase
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Topics |
- Adult
- Aged
- Autoantibodies
(immunology)
- Female
- Glycine-tRNA Ligase
(immunology)
- Humans
- Lung
(diagnostic imaging, immunology, pathology)
- Male
- Middle Aged
- Myositis
(diagnostic imaging, immunology, pathology)
- Radiography
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