@article {Elhaiannrheumdis-2017-211448, author = {Muriel Elhai and Christophe Meune and Marouane Boubaya and J{\'e}r{\^o}me Avouac and Eric Hachulla and Alexandra Balbir-Gurman and Gabriela Riemekasten and Paolo Air{\`o} and Beatriz Joven and Serena Vettori and Franco Cozzi and Susanne Ullman and L{\'a}szl{\'o} Czirj{\'a}k and Mohammed Tikly and Ulf M{\"u}ller-Ladner and Paola Caramaschi and Oliver Distler and Florenzo Iannone and Lidia P Ananieva and Roger Hesselstrand and Radim Becvar and Armando Gabrielli and Nemanja Damjanov and Maria J Salvador and Valeria Riccieri and Carina Mihai and Gabriella Sz{\"u}cs and Ulrich A Walker and Nicolas Hunzelmann and Duska Martinovic and Vanessa Smith and Carolina de Souza M{\"u}ller and Carlo Maurizio Montecucco and Daniela Opris and Francesca Ingegnoli and Panayiotis G Vlachoyiannopoulos and Bojana Stamenkovic and Edoardo Rosato and Stefan Heitmann and J{\"o}rg H W Distler and Thierry Zenone and Matthias Seidel and Alessandra Vacca and Ellen De Langhe and Srdan Novak and Maurizio Cutolo and Luc Mouthon and J{\"o}rg Henes and Carlo Chizzolini and Carlos Alberto von M{\"u}hlen and Kamal Solanki and Simona Rednic and Lisa Stamp and Branimir Anic and Vera Ortiz Santamaria and Maria De Santis and Sule Yavuz and Walter Alberto Sifuentes-Giraldo and Emmanuel Chatelus and Jiri Stork and Jacob van Laar and Esthela Loyo and Paloma Garc{\'\i}a de la Pe{\~n}a Lefebvre and Kilian Eyerich and Vanesa Cosentino and Juan Jose Alegre-Sancho and Otylia Kowal-Bielecka and Gr{\'e}goire Rey and Marco Matucci-Cerinic and Yannick Allanore}, editor = {,}, title = {Mapping and predicting mortality from systemic sclerosis}, elocation-id = {annrheumdis-2017-211448}, year = {2017}, doi = {10.1136/annrheumdis-2017-211448}, publisher = {BMJ Publishing Group Ltd}, abstract = {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{\textquoteright} survival.}, issn = {0003-4967}, URL = {https://ard.bmj.com/content/early/2017/08/22/annrheumdis-2017-211448}, eprint = {https://ard.bmj.com/content/early/2017/08/22/annrheumdis-2017-211448.full.pdf}, journal = {Annals of the Rheumatic Diseases} }