Mapping and predicting mortality from systemic sclerosis

Journal article

Publication Details

Author(s): 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
Journal: Annals of the Rheumatic Diseases
Publication year: 2017
ISSN: 0003-4967


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.

FAU Authors / FAU Editors

Distler, Jörg PD Dr.
Heisenberg-Professur für Molekulare Mechanismen der Organfibrose

External institutions with authors

Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia
Bispebjerg Hospital
Carol Davila University of Medicine and Pharmacy / Universitatea de Medicină și Farmacie „Carol Davila” (UMF București)
Centro Hospitalar e Universitário de Coimbra (CHUC)
Evolent Health, Inc.
Geneva University Hospitals / Hôpitaux universitaires de Genève (HUG)
Hôpital Cochin
Hôpitaux universitaires de Strasbourg (HUS) / University Hospital Strasbourg
Hospital Asil de Granollers
Hospital Clínico Universitario de Valencia
Hospital Jose Maria Cabral Y Baez
Hospital Universitario 12 de Octubre
Hospital Universitario HM Sanchinarro
Hospital Universitario Ramón y Cajal
Institut Niška Banja
Istituto Clinico Humanitas
Iuliu Hațieganu University of Medicine and Pharmacy / Universitatea de Medicină și Farmacie "Iuliu Hațieganu" (UMF Cluj)
James Cook University Hospital
Justus-Liebig-Universität Gießen
KBC Rijeka
Lund University / Lunds universitet
Marienhospital Stuttgart
Marmara University
National and Kapodistrian University of Athens
National Institute for Health and Medical Research / Institut national de la santé et de la recherche médicale (INSERM)
Policlinico San Matteo Pavia Fondazione IRCCS
Technion - Israel Institute of Technology
Technische Universität München (TUM)
Universidad de Buenos Aires (UBA) / University of Buenos Aires
Universidade Federal do Paraná (UFPR)
Università degli studi della Campania Luigi Vanvitelli
Università degli Studi di Cagliari
Università degli Studi di Firenze / University of Florence
Università degli studi di Milano
Università degli studi "La Sapienza"
Università Politecnica delle Marche (UNIVPM)
Universität Basel
Universitätsklinikum Bonn
Universitätsklinikum Heidelberg
Universitätsklinikum Köln
Universitätsspital Zürich (USZ)
Universität zu Lübeck
Universiteit Gent (UGent) / Ghent University
University Hospital Leuven (UZ) / Universitaire ziekenhuizen Leuven
University Hospital Split
University of Bari Aldo Moro / Università degli Studi di Bari Aldo Moro
University of Belgrade / Универзитет у Београду
University of Białystok
University of Debrecen / Debreceni Egyetem
University of Genova / Università degli Studi di Genova
University of Lille Nord de France / Université Lille Nord de France / Université Lille II
University of Otago
University of Padua
University of Paris 13 - Paris-Nord / Université Paris XIII Paris-Nord
University of Paris 5 - René Descartes / Université Paris V René Descartes
University of Pécs
University of the Witwatersrand (WITS)
University of Verona / Università degli Studi di Verona
University of Waikato
Univerzita Karlova v Praze / Charles University in Prague
V.A. Nasonova Research Institute of Rheumatology
Zagreb University Hospital Centre / Klinički bolnički centar Zagreb (KBC)

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).


Last updated on 2018-09-10 at 07:24