ObjectiveThe diagnosis in the studies analyzing human leucocyte antigen (HLA) of dermatomyositis was based on a combined clinical category of polymyositis/dermatomyositis. This retrospective study investigated the associations of HLA with five dermatomyositis-specific autoantibodies in Japanese patients diagnosed by muscle pathology.MethodsWe diagnosed Japanese patients with dermatomyositis based on sarcoplasmic expression of myxovirus resistance protein A. These patients underwent investigation for five dermatomyositis-specific autoantibodies and HLA genotyping.ResultsOf 175 patients (M:F=83:92; range 1–86 years; mean 46 years), 173 (98.9%) had one of the five autoantibodies. Seven alleles—A*02:07, B*46:01, DRB1*04:07, DRB1*07:01, DRB1*08:03, DQB1*06:01,andDPB1*02:02— were more frequently detected in the patients with dermatomyositis than healthy controls, but these associations were not significant after multiple testing correction. Stratifying by dermatomyositis-specific autoantibodies, we found the associations of already known six and new seven alleles—B*48:01, B*52:01, C*12:02, DRB1*04:05, DRB1*15:02, DPB1*05:01,andDPB1*09:01— with subsets of dermatomyositis. Moreover, significant associations of five alleles with anti-nucleosome remodeling deacetylase complex (Mi-2) remained after multiple testing correction. In particular, theDRB1*04:07(OR=28.9, correctedp=2.7×10-6) andDQB1*06:01(OR=4.0, correctedp=1.6×10-4) alleles were significantly more prevalent in patients with anti-Mi-2 antibody than in controls.ConclusionThis study demonstrated dermatomyositis-specific autoantibodies defined immunogenetic subsets of dermatomyositis.