The pulmonary histopathologic manifestations of the anti-Jo-1 tRNA synthetase syndrome.

Abstract

Of the idiopathic inflammatory myopathies, the anti-aminoacyl tRNA synthetase syndrome has the greatest association with interstitial lung disease (ILD). We reviewed 13 open surgical lung biopsies, four autopsies, and three native lungs resected at transplantation, for pulmonary ILD associated with the presence of anti-histidyl tRNA synthetase (anti-Jo-1) autoantibodies. Fifty percent (N=10) of patients presented with an acute decompensation of pulmonary function manifested as diffuse alveolar damage, although in five patients (25%) this marked diminution in function was superimposed on an underlying chronic interstitial pneumonia (usual interstitial pneumonia (three); nonspecific interstitial pneumonia(two)). Seven (35%) patients had usual interstitial pneumonia and two (10%) had nonspecific interstitial pneumonia exclusively, whereas one patient presented with an organizing pneumonia (5%). This study is the first to highlight the high biopsy incidence of diffuse alveolar damage in this patient population both de novo and superimposed on underlying chronic ILD, and also shows that usual interstitial pneumonia remains a significant pattern of interstitial injury in this autoimmune group. On the basis of coexisting patterns of lung injury, this study also suggests that nonspecific interstitial pneumonia in connective tissue disorders may progress over time to a usual interstitial pneumonia pattern of fibrosis, an observation that could be better assessed with future inclusion of autopsy and transplanted native lungs in study groups.

• Authors: Samuel A Yousem, Kevin Gibson, Naftali Kaminski, Chester V Oddis, Dana P Ascherman
• Journal: Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc (Mod Pathol) Vol. 23 Issue 6 Pg. 874-80 (Jun 2010) ISSN: 1530-0285 [Electronic] United States
• PMID: 20228783 (Publication Type: Journal Article)

Chemical References

• Antibodies, Antinuclear
• Jo-1 antibody
• Histidine-tRNA Ligase

Topics

• Adult
• Aged
• Antibodies, Antinuclear (analysis)
• Autopsy
• Biopsy
• Disease Progression
• Female
• Histidine-tRNA Ligase (immunology)
• Humans
• Lung (immunology, pathology, physiopathology)
• Lung Diseases, Interstitial (immunology, pathology, physiopathology)
• Male
• Middle Aged
• Pennsylvania
• Pulmonary Alveoli (pathology)
• Pulmonary Fibrosis (immunology, pathology)
• Registries
• Retrospective Studies
• Syndrome
• Time Factors