Anti‐OJ and anti‐Th/To antibodies are rare and specific antibodies that are seen in patients with polymyositis/dermatomyositis and systemic sclerosis (SSc), respectively. Anti‐Th/To antibody‐positive scleroderma is associated with severe pulmonary hypertension, interstitial pneumonia, and renal crisis, but is usually not complicated by myositis. Herein, we present the case of a patient with SSc who subsequently developed polymyositis and fulminant cardiomyopathy, and had both anti‐OJ and anti‐Th/To antibodies.
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