Cardiac involvement in systemic sclerosis: mechanisms, manifestations and management

Cardiovascular disease remains a leading cause of mortality in systemic sclerosis (SSc). This review summarizes key mechanisms in the pathophysiology of SSc primary heart involvement (SSc-PHI), presents the main clinical manifestations and their management and discusses unmet needs in SSc-PHI with potential solutions. The pathophysiology of SSc-PHI involves both vascular and immune-mediated pathways, exacerbated by the NLRP3 inflammasome and pro-fibrotic cytokines, leading to myocardial fibrosis as the main cardiac manifestation. Regular screening is recommended to detect SSc-PHI, incorporating clinical assessments, cardiac biomarkers and transthoracic echocardiography. Cardiovascular magnetic resonance imaging (CMR) enables detection of subclinical cardiac involvement, with certain CMR phenotypes holding prognostic significance. Early identification of subclinical disease is a critical unmet need to enhance risk stratification and improve outcomes. Emerging programs and cardiology-rheumatology collaboration will enhance interdisciplinary care and diagnostic strategies.