TY - JOUR TI - Mapping and predicting mortality from systemic sclerosis AU - Elhai, Muriel AU - Meune, Christophe AU - Boubaya, Marouane AU - Avouac, Jérôme AU - Hachulla, Eric AU - Balbir-Gurman, Alexandra AU - Riemekasten, Gabriela AU - Airò, Paolo AU - Joven, Beatriz AU - Vettori, Serena AU - Cozzi, Franco AU - Ullman, Susanne AU - Czirják, László AU - Tikly, Mohammed AU - Müller-Ladner, Ulf AU - Caramaschi, Paola AU - Distler, Oliver AU - Iannone, Florenzo AU - Ananieva, Lidia P. AU - Hesselstrand, Roger AU - Becvar, Radim AU - Gabrielli, Armando AU - Damjanov, Nemanja AU - Salvador, Maria J. AU - Riccieri, Valeria AU - Mihai, Carina AU - Szücs, Gabriella AU - Walker, Ulrich A. AU - Hunzelmann, Nicolas AU - Martinovic, Duska AU - Smith, Vanessa AU - Müller, Carolina de Souza AU - Montecucco, Carlo Maurizio AU - Opris, Daniela AU - Ingegnoli, Francesca AU - Vlachoyiannopoulos, Panayiotis G. AU - Stamenkovic, Bojana AU - Rosato, Edoardo AU - Heitmann, Stefan AU - Distler, Jörg H. W. AU - Zenone, Thierry AU - Seidel, Matthias AU - Vacca, Alessandra AU - Langhe, Ellen De AU - Novak, Srdan AU - Cutolo, Maurizio AU - Mouthon, Luc AU - Henes, Jörg AU - Chizzolini, Carlo AU - Mühlen, Carlos Alberto von AU - Solanki, Kamal AU - Rednic, Simona AU - Stamp, Lisa AU - Anic, Branimir AU - Santamaria, Vera Ortiz AU - Santis, Maria De AU - Yavuz, Sule AU - Sifuentes-Giraldo, Walter Alberto AU - Chatelus, Emmanuel AU - Stork, Jiri AU - Laar, Jacob van AU - Loyo, Esthela AU - García de la Peña Lefebvre, Paloma AU - Eyerich, Kilian AU - Cosentino, Vanesa AU - Alegre-Sancho, Juan Jose AU - Kowal-Bielecka, Otylia AU - Rey, Grégoire AU - Matucci-Cerinic, Marco AU - Allanore, Yannick AU - EUSTAR group T2 - Annals of the Rheumatic Diseases AB - 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. DA - 2017/08/23/ PY - 2017 DO - 10.1136/annrheumdis-2017-211448 DP - PubMed J2 - Ann. Rheum. Dis. LA - eng SN - 1468-2060 KW - cardiovascular disease KW - pulmonary fibrosis KW - systemic sclerosis ER -