Heart and systemic sclerosis—findings from a national cohort study
Alexis F. Guédon, Fabrice Carrat, Luc Mouthon, David Launay, Benjamin Chaigne, G. Pugnet, Jean‐Christophe Lega, A. Hot, Vincent Cottin, C. Agard, Yannick Allanore, A.L. Fauchais, P. Jégo, Robin Dhôte, T. Papo, Emmanuel Chatelus, Bernard Bonnotte, Jean-Emmanuel Khan, Élisabeth Diot, Boris Bienvenu, N. Magy‐Bertrand, V. Queyrel, Alain Le Quellec, P. Kieffer, Zahir Amoura, Jean‐Robert Harlé, Jean-Baptiste Gaultier, Marie-Hélène Balquet, Denis Wahl, Olivier Lidove, Olivier Fain, A. Mékinian, É. Hachulla, Sébastien Rivière
Abstract
OBJECTIVES: Heart involvement is one of the leading causes of death in SSc. The prevalence of SSc-related cardiac involvement is poorly known. Our objective was to investigate the prevalence and prognosis burden of different heart diseases in a nationwide cohort of patients with SSc. METHODS: We used data from a multicentric prospective study using the French SSc national database. Focusing on SSc-related cardiac involvement, we aimed to determine its incidence and risk factors. RESULTS: Of the 3528 patients with SSc, 312 (10.9%) had SSc-related cardiac involvement at baseline. They tended to have a diffuse SSc subtype more frequently and to have more severe clinical features, and presented more cardiovascular risk factors. From the 1646 patients available for follow-up analysis, SSc-related cardiac involvement was associated with an increased risk of death. There was no significant difference in overall survival between SSc-related cardiac involvement, ischaemic heart disease or pulmonary arterial hypertension. Regarding survival analysis, 98 patients developed SSc-related cardiac involvement at 5 years (5-year event rate 11.15%). Regarding reduced left ventricular ejection fraction <50% and left ventricular diastolic dysfunction, the 5-year event rate was 2.49% and 5.84%, respectively. Pericarditis cumulative incidence at 5 years was 3%. Diffuse SSc subtype was a risk factor for SSc-related cardiac involvement and pericarditis. Female sex was associated with less left ventricular diastolic dysfunction incidence. CONCLUSIONS: Our results describe the incidence and prognostic burden of SSc-related cardiac involvement at a large scale, with gender and diffuse SSc subtype as risk factors. Further analyses should assess the potential impact of treatment on these various cardiac outcomes.