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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.
Keywords
EULAR SCLERODERMA TRIALS, EUSTAR DATABASE, 5-YEAR SURVIVAL, COHORT, MODEL, METAANALYSIS, DEATH, POPULATION, VALIDATION, PROFILE

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Chicago
Elhai, Muriel, Christophe Meune, Marouane Boubaya, Jérôme Avouac, Eric Hachulla, Alexandra Balbir-Gurman, Gabriela Riemekasten, et al. 2017. “Mapping and Predicting Mortality from Systemic Sclerosis.” Annals of the Rheumatic Diseases 76 (11): 1897–1905.
APA
Elhai, M., Meune, C., Boubaya, M., Avouac, J., Hachulla, E., Balbir-Gurman, A., Riemekasten, G., et al. (2017). Mapping and predicting mortality from systemic sclerosis. ANNALS OF THE RHEUMATIC DISEASES, 76(11), 1897–1905.
Vancouver
1.
Elhai M, Meune C, Boubaya M, Avouac J, Hachulla E, Balbir-Gurman A, et al. Mapping and predicting mortality from systemic sclerosis. ANNALS OF THE RHEUMATIC DISEASES. 2017;76(11):1897–905.
MLA
Elhai, Muriel et al. “Mapping and Predicting Mortality from Systemic Sclerosis.” ANNALS OF THE RHEUMATIC DISEASES 76.11 (2017): 1897–1905. Print.
@article{8543873,
  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.},
  author       = {Elhai, Muriel and Meune, Christophe and Boubaya, Marouane and Avouac, Jérôme and Hachulla, Eric and Balbir-Gurman, Alexandra and Riemekasten, Gabriela and Airò, Paolo and Joven, Beatriz and Vettori, Serena and Cozzi, Franco and Ullman, Susanne and Czirják, László and Tikly, Mohammed and Müller-Ladner, Ulf and Caramaschi, Paola and Distler, Oliver and Iannone, Florenzo and Ananieva, Lidia P and Hesselstrand, Roger and Becvar, Radim and Gabrielli, Armando and Damjanov, Nemanja and Salvador, Maria J and Riccieri, Valeria and Mihai, Carina and Szücs, Gabriella and Walker, Ulrich A and Hunzelmann, Nicolas and Martinovic, Duska and Smith, Vanessa and Müller, Carolina de Souza and Montecucco, Carlo Maurizio and Opris, Daniela and Ingegnoli, Francesca and Vlachoyiannopoulos, Panayiotis G and Stamenkovic, Bojana and Rosato, Edoardo and Heitmann, Stefan and Distler, Jörg HW and Zenone, Thierry and Seidel, Matthias and Vacca, Alessandra and De Langhe, Elle and Novak, Srdan and Cutolo, Maurizio and Mouthon, Luc and Henes, Jörg and Chizzolini, Carlo and von Mühlen, Carlos Alberto and Solanki, Kamal and Rednic, Simona and Stamp, Lisa and Anic, Branimir and Santamaria, Vera Ortiz and De Santis, Maria and Yavuz, Sule and Sifuentes-Giraldo, Walter Alberto and Chatelus, Emmanuel and Stork, Jiri and van Laar, Jacob and Loyo, Esthela and García de la Peña Lefebvre, Paloma and Eyerich, Kilian and Cosentino, Vanesa and Alegre-Sancho, Juan Jose and Kowal-Bielecka, Otylia and Rey, Grégoire and Matucci-Cerinic, Marco and Allanore, Yannick},
  issn         = {0003-4967},
  journal      = {ANNALS OF THE RHEUMATIC DISEASES},
  keywords     = {EULAR SCLERODERMA TRIALS,EUSTAR DATABASE,5-YEAR SURVIVAL,COHORT,MODEL,METAANALYSIS,DEATH,POPULATION,VALIDATION,PROFILE},
  language     = {eng},
  number       = {11},
  pages        = {1897--1905},
  title        = {Mapping and predicting mortality from systemic sclerosis},
  url          = {http://dx.doi.org/10.1136/annrheumdis-2017-211448},
  volume       = {76},
  year         = {2017},
}

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