@article{3000964,
    title = "Mapping and predicting mortality from systemic sclerosis",
    author = "Elhai, M. and Meune, C. and Boubaya, M. and Avouac, J. and Hachulla, E. and Balbir-Gurman, A. and Riemekasten, G. and Airò, P. and Joven, B. and Vettori, S. and Cozzi, F. and Ullman, S. and Czirják, L. and Tikly, M. and Müller-Ladner, U. and Caramaschi, P. and Distler, O. and Iannone, F. and Ananieva, L.P. and Hesselstrand, R. and Becvar, R. and Gabrielli, A. and Damjanov, N. and Salvador, M.J. and Riccieri, V. and Mihai, C. and Szücs, G. and Walker, U.A. and Hunzelmann, N. and Martinovic, D. and Smith, V. and Müller, C.D.S. and Montecucco, C.M. and Opris, D. and Ingegnoli, F. and Vlachoyiannopoulos, P.G. and Stamenkovic, B. and Rosato, E. and Heitmann, S. and Distler, J.H.W. and Zenone, T. and Seidel, M. and Vacca, A. and Langhe, E.D. and Novak, S. and Cutolo, M. and Mouthon, L. and Henes, J. and Chizzolini, C. and Mühlen, C.A.V. and Solanki, K. and Rednic, S. and Stamp, L. and Anic, B. and Santamaria, V.O. and Santis, M.D. and Yavuz, S. and Sifuentes-Giraldo, W.A. and Chatelus, E. and Stork, J. and Laar, J.V. and Loyo, E. and De La Peña Lefebvre, P.G. and Eyerich, K. and Cosentino, V. and Alegre-Sancho, J.J. and Kowal-Bielecka, O. and Rey, G. and Matucci-Cerinic, M. and Allanore, Y.",
    journal = "Annals of the Rheumatic Diseases",
    year = "2017",
    volume = "76",
    number = "11",
    pages = "1897-1905",
    publisher = "BMJ Publishing Group",
    issn = "0003-4967, 1468-2060",
    doi = "10.1136/annrheumdis-2017-211448",
    keywords = "adult;  aged;  all cause mortality;  Article;  cause of death;  data base;  death certificate;  disease association;  female;  follow up;  Frenchman;  heart disease;  human;  major clinical study;  male;  middle aged;  mortality;  mortality rate;  patient monitoring;  prediction;  priority journal;  prognosis;  respiratory tract disease;  risk factor;  survival rate;  systemic sclerosis;  validation study;  very elderly;  factual database;  France;  proportional hazards model;  systemic sclerosis;  time factor, Aged;  Cause of Death;  Databases, Factual;  Death Certificates;  Female;  France;  Humans;  Male;  Middle Aged;  Prognosis;  Proportional Hazards Models;  Risk Factors;  Scleroderma, Systemic;  Time Factors",
    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' survival. ©Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted."
}