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Summary: Background: Myositis‐specific autoantibodies (MSAs) are associated with unique clinical subsets in polymyositis/dermatomyositis (PM/DM). Autoantibodies against transcriptional intermediary factor (TIF)‐1γ and TIF‐1α are known to be MSAs. Previously, we reported that TIF‐1β is also targeted in patients with DM with or without concomitant anti‐TIF‐1α/γ antibodies. Objectives: To evaluate the clinical features of seven cases with anti‐TIF‐1β antibodies alone. Methods: Serum autoantibody profiles were determined, and protein and RNA immunoprecipitation studies were conducted. Western blotting was performed to confirm autoantibody reactivity against TIF‐1β. Results: Anti‐TIF‐1β antibody was identified by immunoprecipitation assay in 24 cases. Among them, seven patients were positive for anti‐TIF‐1β antibody alone. Six of the seven patients were classified as having DM. Among the six cases of DM, two patients had no muscle weakness and normal creatine kinase (CK) levels, and were classified as having clinically amyopathic DM. Four patients had muscle weakness, but three of them had normal serum CK levels that responded well to systemic steroids. Characteristic features of DM included skin rashes, such as Gottron sign, periungual erythema, punctate haemorrhage on the perionychium and facial erythema including heliotrope, which were observed in 86%, 57%, 86% and 71% of our cases, respectively. One of the seven patients had appendiceal cancer. None of the patients had interstitial lung disease. Conclusions: Seven patients were confirmed to have anti‐TIF‐1β antibody without any other MSAs, including TIF‐1α/γ antibodies, and six of them were diagnosed with DM. We suggest that anti‐TIF‐1β antibody is an MSA, and that it is associated with clinically amyopathic DM or DM with mild myopathy. What's already known about this topic? Previously we reported that transcriptional intermediary factor (TIF)‐1β is also targeted in patients with dermatomyositis with or without concomitant anti‐TIF‐1α/γ antibodies.Anti‐TIF‐1β antibody could be a myositis‐specific autoantibody. What does this study add? We describe seven patients with anti‐TIF‐1β antibody but without anti‐TIF‐1α/γ reactivity.Anti‐TIF‐1β antibody is possibly associated with clinically amyopathic dermatomyositis or dermatomyositis with mild myopathy. Linked Comment:Fiorentino. Br J Dermatol 2019; 180:709–710. Respond to this article Plain language summary available online [ABSTRACT FROM AUTHOR]