Mapping and predicting mortality from systemic sclerosis

Elhai M, Meune C, Boubaya M, Avouac J, Hachulla E, Balbir-Gurman A, Riemekasten G, Airo P, Joven B, Vettori S, Cozzi F, Ullman S, Czirjak L, Tikly M, Mueller-Ladner ULF, Caramaschi P, Distler O, Iannone F, Ananieva LP, Hesselstrand R, Becvar R, Gabrielli A, Damjanov N, Salvador MJ, Riccieri V, Mihai C, Szucs G, Walker UA, Hunzelmann N, Martinovic D, Smith V, Mueller CDS, Montecucco CM, Opris D, Ingegnoli F, Vlachoyiannopoulos PG, Stamenkovic B, Rosato E, Heitmann S, Distler J, Zenone T, Seidel M, Vacca A, De Langhe E, Novak S, Cutolo M, Mouthon L, Henes J, Chizzolini C, Von Muhlen CA, Solanki K, Rednic S, Stamp L, Anic B, Santamaria VO, De Santis M, Yavuz S, Alberto Sifuentes-Giraldo W, Chatelus E, Stork J, Van Laar J, Loyo E, De La Pena Lefebvre PG, Eyerich K, Cosentino V, Jose Alegre-Sancho J, Kowal-Bielecka O, Rey G, Matucci-Cerinic M, Allanore Y (2017)

Publication Type: Journal article

Publication year: 2017


DOI: 10.1136/annrheumdis-2017-211448


To determine the causes of death and risk factors in systemic sclerosis (SSc).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.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.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.

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Université de Lille (ULille) / University of Lille Nord de France / Université Lille Nord de France / Université Lille II FR France (FR) University of Paris 5 - René Descartes / Université Paris V René Descartes FR France (FR) University of Paris 13 - Paris-Nord / Université Paris XIII Paris-Nord FR France (FR) Technion - Israel Institute of Technology IL Israel (IL) Universität zu Lübeck DE Germany (DE) Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia IT Italy (IT) Hospital Universitario 12 de Octubre ES Spain (ES) Università degli studi della Campania Luigi Vanvitelli IT Italy (IT) University of Padua / Universita degli Studi di Padova IT Italy (IT) Bispebjerg Hospital DK Denmark (DK) University of Pécs / Pécsi Tudományegyetem HU Hungary (HU) University of the Witwatersrand (WITS) ZA South Africa (ZA) Justus-Liebig-Universität Gießen DE Germany (DE) University of Verona / Università degli Studi di Verona IT Italy (IT) Universitätsspital Zürich (USZ) CH Switzerland (CH) University of Bari Aldo Moro / Università degli Studi di Bari Aldo Moro IT Italy (IT) V.A. Nasonova Research Institute of Rheumatology RU Russian Federation (RU) Lund University / Lunds universitet SE Sweden (SE) Univerzita Karlova v Praze / Charles University in Prague CZ Czech Republic (CZ) Università Politecnica delle Marche (UNIVPM) / Marche Polytechnic University IT Italy (IT) University of Belgrade / Универзитет у Београду RS Serbia (RS) Centro Hospitalar e Universitário de Coimbra (CHUC) PT Portugal (PT) Università degli studi "La Sapienza" IT Italy (IT) Carol Davila University of Medicine and Pharmacy / Universitatea de Medicină și Farmacie „Carol Davila” (UMF București) RO Romania (RO) University of Debrecen / Debreceni Egyetem HU Hungary (HU) Universität Basel CH Switzerland (CH) Universitätsklinikum Köln DE Germany (DE) University Hospital Split HR Croatia (HR) Universiteit Gent (UGent) / Ghent University BE Belgium (BE) Universidade Federal do Paraná (UFPR) BR Brazil (BR) Policlinico San Matteo Pavia Fondazione IRCCS IT Italy (IT) Università degli studi di Milano IT Italy (IT) National and Kapodistrian University of Athens GR Greece (GR) Institut Niška Banja RS Serbia (RS) Marienhospital Stuttgart DE Germany (DE) Evolent Health, Inc. US United States (USA) (US) Universitätsklinikum Bonn DE Germany (DE) Università degli Studi di Cagliari IT Italy (IT) KBC Rijeka HR Croatia (HR) University of Genova / Università degli Studi di Genova IT Italy (IT) Hôpital Cochin FR France (FR) Universitätsklinikum Heidelberg DE Germany (DE) Geneva University Hospitals / Hôpitaux universitaires de Genève (HUG) CH Switzerland (CH) University of Waikato NZ New Zealand (NZ) Iuliu Hațieganu University of Medicine and Pharmacy / Universitatea de Medicină și Farmacie "Iuliu Hațieganu" (UMF Cluj) RO Romania (RO) University of Otago NZ New Zealand (NZ) Zagreb University Hospital Centre / Klinički bolnički centar Zagreb (KBC) HR Croatia (HR) Hospital Asil de Granollers ES Spain (ES) Istituto Clinico Humanitas IT Italy (IT) Marmara University TR Turkey (TR) Hospital Universitario Ramón y Cajal ES Spain (ES) Hôpitaux universitaires de Strasbourg (HUS) / University Hospital Strasbourg FR France (FR) James Cook University Hospital GB United Kingdom (GB) Hospital Jose Maria Cabral Y Baez DO Dominican Republic (DO) Hospital Universitario HM Sanchinarro ES Spain (ES) Technische Universität München (TUM) DE Germany (DE) Universidad de Buenos Aires (UBA) / University of Buenos Aires AR Argentina (AR) University Hospital Leuven (UZ) / Universitaire ziekenhuizen Leuven BE Belgium (BE) Hospital Clínico Universitario de Valencia ES Spain (ES) University of Białystok PL Poland (PL) National Institute for Health and Medical Research / Institut national de la santé et de la recherche médicale (INSERM) FR France (FR) Università degli Studi di Firenze / University of Florence IT Italy (IT)

How to cite


Elhai, M., Meune, C., Boubaya, M., Avouac, J., Hachulla, E., Balbir-Gurman, A.,... Allanore, Y. (2017). Mapping and predicting mortality from systemic sclerosis. Annals of the Rheumatic Diseases.


Elhai, Muriel, et al. "Mapping and predicting mortality from systemic sclerosis." Annals of the Rheumatic Diseases (2017).

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