Cardiac disease in systemic sclerosis: Integrating pathobiology with clinical management

Abstract Systemic sclerosis (SSc) is a complex autoimmune disorder in which cardiovascular involvement remains a major determinant of morbidity and mortality. Cardiac injury in SSc results from the interplay of microvascular dysfunction, immune‐mediated inflammation, and progressive interstitial and replacement fibrosis, leading to myocardial disease, arrhythmias, pericardial abnormalities, and coronary microvascular ischemia. Many of these manifestations evolve silently, becoming clinically apparent only after substantial structural or electrical remodeling has occurred, highlighting the critical need for systematic surveillance. Current consensus algorithms recommend a core annual cardiovascular assessment—including symptom‐directed clinical evaluation, electrocardiography, transthoracic echocardiography with tissue Doppler and strain imaging, and measurement of natriuretic peptides and high‐sensitivity troponin—with escalation to cardiac magnetic resonance imaging, ambulatory rhythm monitoring, or stress testing when abnormalities are detected. Management integrates standard heart failure and arrhythmia therapies with judicious use of immunomodulation in patients with active inflammatory cardiomyopathy. However, evidence for targeted immunosuppressive treatment of primary cardiac involvement in SSc remains limited, and the optimal frequency, modality, and biomarker combinations for screening continue to be refined. This review synthesizes emerging mechanisms, diagnostic strategies, and therapeutic considerations, and outlines research priorities aimed at improving early detection and outcomes in cardiovascular manifestations of SSc.