Clinical manifestation and prognostic factor in anti-aminoacyl-tRNA synthetase autoantibodies-associated interstitial lung disease

Objectives: To compare the clinicopathological characteristics of interstitial lung disease (ILD) patients with anti-Jo-1 antibodies to anti-aminoacyl-tRNA synthetase (anti-ARS) autoantibodies other than anti-Jo-1 antibodies. Methods: We retrospectively investigated clinical findings in 19 ILD patients with anti-ARS autoantibodies between 2007 and 2012. Results: Anti-Jo-1 antibodies were the most common (63.2%, 12/19), followed by anti-PL-7 (15.8%, 3/19), anti-EJ (10.5%, 2/19), and anti-PL-12 and anti-OJ (each 5.3%, 1/19). The 12 patients with anti-Jo-1 antibodies comprised 3 men and 9 women. Mean age was 61.3. Seven patients had definite diagnosis of connective tissue disease (CTD); 6 of whom were polymyositis (PM), and 1 Sjogren syndrome. Two of 7 manifested ILD preceding CTD symptoms. Three patients died during follow-up period; 1 patient with PM died of breast cancer, and 2 died of ILD. Surgical lung biopsy was performed in 2 patients. Results of histopathological findings showed nonspecific interstitial pneumonia (NSIP) pattern and unclassified pattern in each patient. The 7 patients with anti-ARS autoantibodies other than anti-Jo-1 antibodies comprised 1 man and 6 women. Mean age was 64.2. Four patients had definite diagnosis of CTD; 2 of whom were PM, 1 rheumatoid arthritis (RA), and 1 both RA and dermatomyositis (DM). Three of 4 manifested ILD preceding CTD. One patient with PM died of gastric cancer. All of 3 patients who were performed surgical lung biopsy showed NSIP pattern. Conclusion: NSIP was the predominant histopathologic pattern in ILD patients with anti-ARS autoantibodies. PM with cancer was associated with poor survival.