Autoantibodies against aminoacyl-tRNA synthetase (ARS) are known to associate with myositis, arthritis, and interstitial lung disease (ILD). Anti Jo-1 antibody is a common diagnostic tool of this syndrome and is available on a commercial basis, while other anti-ARS antibodies such as anti-EJ, anti-PL7 and anti-OJ antibodies are less commonly examined. The aim of this study was to investigate the clinicopathological characteristics of 9 ILD patients who were positive for anti-ARS antibodies including anti-EJ, anti-PL7 and anti-Jo-1 antibodies. In physical examination, muscle pain and/or skin rash (including mechanic's hand) were observed frequently. In laboratory findings, levels of myogenic enzymes were slightly elevated, and anti-nucleus antibody was detected in most cases. Air-space consolidation with traction bronchiectasis, ground glass opacities and characteristic volume loss of both lower lobes were observed in radiological examination. Organization in alveolar spaces and lymphoid alveolitis that did not destroy existing pulmonary structures were main pathological findings. Immunosupressive therapy was effective and the prognosis appears satisfactory in these cases. Although anti-ARS antibodies had been measured as a marker of myositis, we suggest that they are useful as markers of interstitial pneumonia.