Clinical and radiological features of adult patients with different antisynthetase syndrome based on serum anti-aminoacyl-tRNA synthetase antibody type

Abstract

Objective: To describe the clinical and radiological characteristics of antisynthetase syndrome associated interstitial lung disease in patients with different serum anti-aminoacyl-tRNA synthetase antibodies. Methods: We conducted a retrospective analysis of 5 adult patients with antisynthetase syndrome associated interstitial lung disease in Peking Union Medical College Hospital. Their clinical and chest radiological data were analyzed and relevant literatures were reviewed. Results: Among these 5 patients, there were 1 male and 4 females, aged from 32 y to 67 y, with a mean age of 53 y. Cough and exertional dyspnea were the main clinical complaints. Four cases showed mechanic's-like hands, and all of the 5 cases had Velcro rales in the basal lungs. None of them showed clubbing. Creatine kinase was elevated in 1 case with anti-Jo-1 synthetase antibody, and anti-nuclear antibody was positive in 4 cases, with different titers from 1∶80 to 1∶320, and anti-Ro-52 antibody was positive in 3 cases. Anti-aminoacyl-tRNA synthetase antibody spectrum analysis showed 1 case with anti-Jo-1, 1 anti-PL-7, 1 anti PL-12, 1 anti-EJ and 1 anti-OJ synthetase antibody, respectively. Chest high resolution CT showed nonspecific interstitial pneumonia pattern in 1 case, and nonspecific interstitial pneumonia pattern with organizing pneumonia pattern in 4 cases. All the cases responded to immunosuppressive therapy including corticosteroids (with starting prednisone dosage more than 1 mg·kg(-1)·d(-1)) plus azathioprine or Mycophenolate mofetil. Conclusions: Antisynthetase syndrome associated interstitial lung disease, characterized by the presence of different anti-tRNA synthetase antibodies, is an increasingly recognized clinical entity. Clinical and radiological features of different subtypes of antisynthetase syndrome are relatively heterogeneous. Nonspecific interstitial pneumonia pattern, nonspecific interstitial pneumonia pattern with organizing pneumonia pattern and organizing pneumonia pattern were common chest HRCT patterns. Prednisone plus immunosuppressive agents are the recommended first line therapy.