Abstract
Cardiac involvement in idiopathic inflammatory myopathies is typically subclinical; however, it is an important predictor of poor prognosis. Although data are limited, including two small cohorts and several case reports, evidence has demonstrated that cardiac involvement in anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis is a significant independent predictor of mortality. Here, we summarized the characteristics of two patients with anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis who developed fatal cardiac complications, one at early stage and the other during long-term follow-up of the disease. Despite aggressive treatment of the primary condition and intensive life support, both patients ultimately succumbed to their cardiac events. These cases highlight the importance of cardiac evaluation and monitoring in patients with anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis.