• Frank Schneider, Rohit Aggarwal, David Bi, Kevin Gibson, Chester Oddis, and Samuel A Yousem.
• From the Departments of Pathology (Drs Schneider and Yousem, Mr Bi), Rheumatology (Drs Aggarwal and Oddis), and Pulmonary Medicine (Dr Gibson), UPMC Presbyterian, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.
• Arch. Pathol. Lab. Med. 2015 Jan 1; 139 (1): 122-5.

ContextThe clinical spectrum of the antisynthetase syndromes (AS) has been poorly defined, although some frequently present with pulmonary manifestations. The anti-KS anti-asparaginyl-transfer RNA synthetase syndrome is one in which pulmonary interstitial lung disease is almost always present and yet the histopathologic spectrum is not well described.ObjectiveTo define the morphologic manifestations of pulmonary disease in those patients with anti-KS antiasparaginyl syndrome.DesignWe reviewed the connective tissue disorder registry of the University of Pittsburgh and identified those patients with anti-KS autoantibodies who presented with interstitial lung disease and had surgical lung biopsies.ResultsThe 5 patients with anti-KS antisynthetase syndrome were usually women presenting with dyspnea and without myositis, but with mechanic's hands (60%) and Raynaud phenomenon (40%). They most often presented with a usual interstitial pneumonia pattern of fibrosis (80%), with the final patient displaying organizing pneumonia.ConclusionsPulmonary interstitial lung disease is a common presentation in patients with the anti-KS-antisynthetase syndrome, who are often women with rather subtle or subclinical connective tissue disease, whereas the literature emphasizes the nonspecific interstitial pneumonia pattern often diagnosed clinically. Usual interstitial pneumonia and organizing pneumonia patterns of interstitial injury need to be added to this clinical differential diagnosis.

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