Cardiac disease in systemic sclerosis: a narrative review.

Abstract

Systemic sclerosis (SSc) is an autoimmune disorder causing immune dysregulation, leading to microvascular damage and fibrosis. Cardiovascular involvement begins with endothelial dysfunction and progresses to vasospasm, ischemia, and fibrosis, impairing cardiac function. Common manifestations include myocardial fibrosis, conduction abnormalities, heart failure, and valvular dysfunction, affecting 15-35% of patients and contributing to 27.2% of SSc-related deaths. High-risk factors include male gender, older disease onset, African American ethnicity, diffuse cutaneous SSc, and poor quality of life. Subclinical cardiac dysfunction is common but underdiagnosed, and cardiovascular complications worsen outcomes. Pulmonary arterial hypertension is a major cause of morbidity and mortality and requires early diagnosis and multidisciplinary management. Treatments include calcium channel blockers, phosphodiesterase inhibitors, endothelin receptor antagonists, and prostacyclin analogs. Imaging (echocardiography, cardiac MRI, stress tests) and biomarkers (troponin, NT-proBNP, endothelin-1) help in diagnosis and monitoring. Early recognition and comprehensive management improve patient outcomes.