We describe the presence of anti‐ACE2 IgM antibodies in two separate anti‐MDA5‐positive DM cohorts: approximately 10% (n = 5 of 52) in a largely outpatient North American cohort and 15% (n = 5 of 32) in a Japanese cohort enriched for new‐onset or severe disease. The gene discussed is CD40LG; the disease is dermatomyositis.