The pulmonary histopathologic manifestations of the anti-PL7/antithreonyl transfer RNA synthetase syndrome

Abstract

The pulmonary histopathologic manifestations of the antisynthetase syndromes is poorly understood and reported. Eight cases of interstitial lung disease related to the presence of antitheonyl (PL7) transfer RNA synthetase autoantibodies along with a review of biopsy changes reported in the English literature are described. Most patients presented with dyspnea and cough, with myositis, skin changes including "mechanic's hands," and Raynaud phenomenon. Biopsy patterns of injury included usual interstitial pneumonia (n = 4), organizing pneumonia (n = 2), nonspecific interstitial pneumonia, and lymphoid interstitial pneumonia (n = 1 each), which, in a minority of instances, contrasted with predictions by thoracic radiologists based on high-resolution computed tomographic scans. This study emphasizes the integrated clinical, radiologic, and pathologic approach to interstitial lung disease, especially in connective tissue disorders, and points out the occurrence of usual interstitial pneumonia and organizing pneumonia in this patient group where nonspecific interstitial pneumonia has been the dominant pattern of clinical interest.

Keywords: Antisynthetase syndrome; Autoimmune; Lung; PL7.