TY - JOUR T1 - Mapping and predicting mortality from systemic sclerosis JF - Annals of the Rheumatic Diseases JO - Ann Rheum Dis SP - 1897 LP - 1905 DO - 10.1136/annrheumdis-2017-211448 VL - 76 IS - 11 AU - Muriel Elhai AU - Christophe Meune AU - Marouane Boubaya AU - Jérôme Avouac AU - Eric Hachulla AU - Alexandra Balbir-Gurman AU - Gabriela Riemekasten AU - Paolo Airò AU - Beatriz Joven AU - Serena Vettori AU - Franco Cozzi AU - Susanne Ullman AU - László Czirják AU - Mohammed Tikly AU - Ulf Müller-Ladner AU - Paola Caramaschi AU - Oliver Distler AU - Florenzo Iannone AU - Lidia P Ananieva AU - Roger Hesselstrand AU - Radim Becvar AU - Armando Gabrielli AU - Nemanja Damjanov AU - Maria J Salvador AU - Valeria Riccieri AU - Carina Mihai AU - Gabriella Szücs AU - Ulrich A Walker AU - Nicolas Hunzelmann AU - Duska Martinovic AU - Vanessa Smith AU - Carolina de Souza Müller AU - Carlo Maurizio Montecucco AU - Daniela Opris AU - Francesca Ingegnoli AU - Panayiotis G Vlachoyiannopoulos AU - Bojana Stamenkovic AU - Edoardo Rosato AU - Stefan Heitmann AU - Jörg H W Distler AU - Thierry Zenone AU - Matthias Seidel AU - Alessandra Vacca AU - Ellen De Langhe AU - Srdan Novak AU - Maurizio Cutolo AU - Luc Mouthon AU - Jörg Henes AU - Carlo Chizzolini AU - Carlos Alberto von Mühlen AU - Kamal Solanki AU - Simona Rednic AU - Lisa Stamp AU - Branimir Anic AU - Vera Ortiz Santamaria AU - Maria De Santis AU - Sule Yavuz AU - Walter Alberto Sifuentes-Giraldo AU - Emmanuel Chatelus AU - Jiri Stork AU - Jacob van Laar AU - Esthela Loyo AU - Paloma García de la Peña Lefebvre AU - Kilian Eyerich AU - Vanesa Cosentino AU - Juan Jose Alegre-Sancho AU - Otylia Kowal-Bielecka AU - Grégoire Rey AU - Marco Matucci-Cerinic AU - Yannick Allanore A2 - , Y1 - 2017/11/01 UR - http://ard.bmj.com/content/76/11/1897.abstract N2 - Objectives To determine the causes of death and risk factors in systemic sclerosis (SSc).Methods Between 2000 and 2011, we examined the death certificates of all French patients with SSc to determine causes of death. Then we examined causes of death and developed a score associated with all-cause mortality from the international European Scleroderma Trials and Research (EUSTAR) database. Candidate prognostic factors were tested by Cox proportional hazards regression model by single variable analysis, followed by a multiple variable model stratified by centres. The bootstrapping technique was used for internal validation.Results We identified 2719 French certificates of deaths related to SSc, mainly from cardiac (31%) and respiratory (18%) causes, and an increase in SSc-specific mortality over time. Over a median follow-up of 2.3 years, 1072 (9.6%) of 11 193 patients from the EUSTAR sample died, from cardiac disease in 27% and respiratory causes in 17%. By multiple variable analysis, a risk score was developed, which accurately predicted the 3-year mortality, with an area under the curve of 0.82. The 3-year survival of patients in the upper quartile was 53%, in contrast with 98% in the first quartile.Conclusion Combining two complementary and detailed databases enabled the collection of an unprecedented 3700 deaths, revealing the major contribution of the cardiopulmonary system to SSc mortality. We also developed a robust score to risk-stratify these patients and estimate their 3-year survival. With the emergence of new therapies, these important observations should help caregivers plan and refine the monitoring and management to prolong these patients’ survival. ER -