Mapping and predicting mortality from systemic sclerosis

Muriel Elhai, Christophe Meune, Marouane Boubaya, Jérôme Avouac, Eric Hachulla, Alexandra Balbir-Gurman, Gabriela Riemekasten, Paolo Airò, Beatriz Joven, Serena Vettori, Franco Cozzi, Susanne Ullman, László Czirják, Mohammed Tikly, Ulf Müller-Ladner, Paola Caramaschi, Oliver Distler, Florenzo Iannone, Lidia P. Ananieva, Roger HesselstrandRadim Becvar, Armando Gabrielli, Nemanja Damjanov, Maria J. Salvador, Valeria Riccieri, Carina Mihai, Gabriella Szücs, Ulrich A. Walker, Nicolas Hunzelmann, Duska Martinovic, Vanessa Smith, Carolina De Souza Müller, Carlo Maurizio Montecucco, Daniela Opris, Francesca Ingegnoli, Panayiotis G. Vlachoyiannopoulos, Bojana Stamenkovic, Edoardo Rosato, Stefan Heitmann, Jörg H.W. Distler, Thierry Zenone, Matthias Seidel, Alessandra Vacca, Ellen De Langhe, Srdan Novak, Maurizio Cutolo, Luc Mouthon, Jörg Henes, Carlo Chizzolini, Carlos Alberto Von Mühlen, Kamal Solanki, Simona Rednic, Lisa Stamp, Branimir Anic, Vera Ortiz Santamaria, Maria De Santis, Sule Yavuz, Walter Alberto Sifuentes-Giraldo, Emmanuel Chatelus, Jiri Stork, Jacob Van Laar, Esthela Loyo, Paloma Garcia De La Peña Lefebvre, Kilian Eyerich, Vanesa Cosentino, Juan Jose Alegre-Sancho, Otylia Kowal-Bielecka, Grégoire Rey, Marco Matucci-Cerinic, Yannick Allanore*

*Corresponding author for this work
139 Citations (Scopus)


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.

Original languageEnglish
JournalAnnals of the Rheumatic Diseases
Issue number11
Pages (from-to)1897-1905
Number of pages9
Publication statusPublished - 01.11.2017

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)


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