Anti‐MDA5 antibody‐associated clinically amyopathic dermatomyositis diagnosed after the onset of pulmonary hypertension

Abstract

AbstractIdiopathic inflammatory myopathies (IIM) are a group of highly heterogeneous systemic autoimmune diseases, of which clinically amyopathic dermatomyositis (CADM) is a distinct sub‐type. Pulmonary hypertension (PH) is a life‐threatening medical condition that can occur as a complication of connective tissue diseases. Herein, our report first suggests that PH can develop in CADM. A 48‐year‐old woman came to our hospital due to a 3 months history of facial edema and shortness of breath. Relevant examinations revealed Gottron's sign, normal creatinine kinase levels, elevated levels of mean pulmonary artery pressure, double‐positive anti‐MDA5 and anti‐Ro52 antibodies, and typical pathological changes associated with myositis. The diagnosis of CADM combined with PH was considered. The patient responded well to the immunosuppression therapy and PH‐related drug therapy. We provide further insights that patients with IIM need to undergo regular assessment of PH.