Pos1283 heart and systemic sclerosis – findings from national cohort study

Background Heart involvement is one of the leading causes of death in systemic sclerosis (SSc). Cardiac manifestations are heterogeneous. In particular, prevalence of SSc-related cardiopathy is poorly known due to a lack of consensus in its definition. Objectives Our objective was to investigate the prevalence and prognosis burden of the different heart diseases in a nationwide cohort of patients with SSc. Methods We used data from a national multicentric prospective study using the French SSc national database. We described the characteristics of 3 different types of heart involvement: SSc-related cardiopathy, pulmonary arterial hypertension and ischaemic heart disease. We analyzed overall survival and survival according to the heart disease. Focusing on SSc-related cardiopathy, we aimed to determine its incidence and risk factors. Results Over the 3528 patients with SSc available for baseline analyses 312 (10.9%) had SSc-related cardiopathy at baseline. They tended to have a diffuse SSc subtype more frequently, had more severe clinical features, and presented more cardiovascular risk factors. From the 1646 patients available for follow-up analysis, SSc-related cardiopathy was associated with an increased risk of death. No significant difference of overall survival was found between SSc-related cardiopathy, ischaemic heart disease or pulmonary arterial hypertension. Concerning survival analysis, 98 patients developed SSc-related cardiopathy at 5 years (5-year event-rate: 11.15% [9.01; 13.23]). Regarding reduced LVEF < 50% and left ventricular diastolic dysfunction, the 5-year event-rate were 2.49% [CI95%: 1.13; 3.83] and 5.84% [CI95%: 4.02; 7.62], respectively. The pericarditis cumulative incidence at 5 years was 3% [CI95%: 1.91; 4.08]). Diffuse SSc subtype was a risk factor of SSc-related cardiopathy and pericarditis (adjusted HR: 1.42 [CI95%: 1.02; 1.97], p = 0.04 and adjusted HR: 1.79 [CI95%: 1.06; 3.02], p = 0.03, respectively). Female sex was associated with less diastolic dysfunction incidence (adjusted HR: 0.39 [CI95%: 0.24; 0.63], p = 0.0001). Conclusion Our results further describe at a large scale the incidence and prognostic burden of SSc-related cardiopathy, with gender and diffuse SSc subtype as risk factors. Further analyses should assess the potential impact of treatment on these various cardiac outcomes. References [1]Elhai M, Meune C, Boubaya M, Avouac J, Hachulla E, Balbir-Gurman A, et al. Mapping and predicting mortality from systemic sclerosis. Ann Rheum Dis 2017;76:1897–905. https://doi.org/10.1136/annrheumdis-2017-211448 . [2]Bulkley BH, Ridolfi RL, Salyer WR, Hutchins GM. Myocardial lesions of progressive systemic sclerosis. A cause of cardiac dysfunction. Circulation 1976;53:483–90. https://doi.org/10.1161/01.cir.53.3.483 . [3]Hoogen F van den, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013 Classification Criteria for Systemic Sclerosis: An American College of Rheumatology/European League Against Rheumatism Collaborative Initiative. Arthritis & Rheumatism 2013;65:2737–47. https://doi.org/10.1002/art.38098 . Figure 1. Acknowledgements: NIL. Disclosure of Interests Alexis F. Guedon: None declared, Fabrice Carrat: None declared, Luc Mouthon: None declared, David Launay: None declared, Benjamin Chaigne: None declared, Gregory Pugnet: None declared, Jean-Christophe Lega: None declared, Arnaud Hot: None declared, Robin Dhote: None declared, Thomas Papo: None declared, Emmanuel Chatelus: None declared, Bernard Bonnotte: None declared, Jean-Emmanuel Kahn: None declared, Elisabeth Diot Speakers bureau: ED received fees as speaker in symposium from Boehringer Ingelheim., Boris Bienvenu: None declared, Nadine Magy-Bertrand: None declared, Viviane Queyrel: None declared, Alain Le Quellec: None declared, Pierre Kieffer: None declared, Zahir Amoura: None declared, Jean-Robert Harlé: None declared, Vincent Cottin: None declared, Jean-Baptiste Gaultier: None declared, Marie-Hélène Balquet: None declared, DENIS WAHL: None declared, Olivier Lidove Grant/research support from: OL received several fees for congress travels and experts’ use from Amicus Therapeutics, Sanofi-Genzyme, and Takeda, Anne-Laure Fauchais: None declared, Yannick Allanore: None declared, Patrick Jégo: None declared, Christian Agard: None declared, olivier fain: None declared, Arsene Mekinian Grant/research support from: AM is investigator of CELGENE, ROCHE, CHUGAI founded trials with APHP and Hopital 15-20 promotion; AM received several fees for congress travels and experts’ use from LFB, SANOFI, SHIRE, and CELGENE, Eric Hachulla: None declared, Sebastien RIVIERE: None declared.