Rev Med Interne. 2026 Jun 22:S0248-8663(26)00630-2. doi: 10.1016/j.revmed.2026.06.005. Online ahead of print.
ABSTRACT
Systemic sclerosis (SSc) is a rare systemic disease characterised by progressive fibrosis, vasculopathy, and immune dysregulation, resulting in multiorgan damage. Independently of pulmonary arterial hypertension, cardiac involvement, encompassing myocardial fibrosis, coronary microvascular dysfunction, arrhythmias, conduction disorders, pericardial and valvular disease, and heart failure, represents a frequently underestimated cause of morbidity and mortality. Clinically manifest cardiac disease is observed in 15 to 35% of patients, while subclinical dysfunction is detectable in approximately 70% when advanced screening tools are employed. The annual incidence of sudden cardiac death is estimated at between 1.0% and 3.3%, exceeding the general-population risk by more than tenfold. The pathophysiological framework rests on coronary microvascular dysfunction driven by endothelial injury, ischaemia-reperfusion cycles, and TGF-β-mediated replacement fibrosis, potentiated by autonomic nervous system imbalance and activation of the renin-angiotensin-aldosterone system. Electrocardiographic abnormalities, detectable in 25 to 85% of patients, are independent predictors of mortality, while diastolic dysfunction, present in 18 to 62% of cases, constitutes a robust prognostic marker. Cardiac magnetic resonance imaging has transformed subclinical detection, revealing late gadolinium enhancement fibrosis in the majority of screened patients without a prior cardiovascular diagnosis. Management requires a multidisciplinary approach, integrating SSc-specific adaptation of guideline-directed heart failure therapies, immunosuppression targeting inflammatory and fibrotic pathways, arrhythmia management with implantable devices, and EULAR-recommended cardiovascular risk reduction. This narrative review synthesises current evidence on the epidemiology, pathogenesis, diagnostic evaluation, and therapeutic strategies of cardiac involvement in SSc, highlighting the need for early, systematic, risk-stratified screening and the establishment of dedicated multidisciplinary cardiac teams.
PMID:42331656 | DOI:10.1016/j.revmed.2026.06.005