Rheumatol Int. 2026 May 25;46(6):110. doi: 10.1007/s00296-026-06127-y.
ABSTRACT
Systemic sclerosis (SSc) is a complex autoimmune disease characterized by immune dysregulation, vasculopathy, and progressive fibrosis. Cardiovascular involvement represents a leading cause of mortality, yet remains underrecognized and frequently subclinical, contributing to delayed diagnosis. This review aims to provide an clinically oriented synthesis of the mechanisms linking autoimmunity to cardiovascular dysfunction in SSc, highlighting emerging concepts in risk stratification, early detection, and management. A comprehensive narrative review was conducted using major databases, focusing on pathophysiology, diagnostic strategies, and therapeutic implications of cardiovascular involvement in SSc. Cardiovascular involvement in SSc is frequent, predominantly subclinical, and driven by immune mediated vasculopathy and myocardial fibrosis, leading to a higher incidence of adverse cardiovascular events compared with the general population. Diastolic dysfunction emerges as the most common and prognostically relevant cardiac phenotype, while systolic dysfunction, pulmonary arterial hypertension-related right ventricular failure, and arrhythmias further contribute to excess mortality. Traditional risk factors alone do not account for this burden; SSc specific features (disease subset, autoantibody profile, interstitial lung disease and microvascular damage) substantially modulate cardiovascular risk and clinical expression. Multimodal imaging, particularly echocardiography, coupled with circulating biomarkers such as NT proBNP, troponin and emerging fibrosis related markers, allows earlier detection of subclinical myocardial involvement, refined stratification and improved prognostic assessment, supporting a structured, risk adapted screening strategy in SSc. This review highlights the need to reconceptualize cardiovascular risk in SSc as a disease-specific entity driven by immune-mediated vasculopathy rather than conventional atherosclerotic paradigms. Current gaps in standardized screening and risk models underscore the necessity for integrated, disease-adapted approaches. Early identification of cardiovascular involvement through systematic screening and multimodal assessment is critical to improving outcomes in SSc. Future research should prioritize the development of validated, SSc-specific cardiovascular risk stratification tools and targeted therapeutic strategies.
PMID:42184052 | DOI:10.1007/s00296-026-06127-y