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. 2017 Nov;76(11):1897-1905.
doi: 10.1136/annrheumdis-2017-211448. Epub 2017 Aug 23.

Mapping and predicting mortality from systemic sclerosis

Muriel Elhai  1 , Christophe Meune  2 , Marouane Boubaya  3 , Jérôme Avouac  1 , Eric Hachulla  4 , Alexandra Balbir-Gurman  5 , Gabriela Riemekasten  6 , Paolo Airò  7 , Beatriz Joven  8 , Serena Vettori  9 , Franco Cozzi  10 , Susanne Ullman  11 , László Czirják  12 , Mohammed Tikly  13 , Ulf Müller-Ladner  14 , Paola Caramaschi  15 , Oliver Distler  16 , Florenzo Iannone  17 , Lidia P Ananieva  18 , Roger Hesselstrand  19 , Radim Becvar  20 , Armando Gabrielli  21 , Nemanja Damjanov  22 , Maria J Salvador  23 , Valeria Riccieri  24 , Carina Mihai  25 , Gabriella Szücs  26 , Ulrich A Walker  27 , Nicolas Hunzelmann  28 , Duska Martinovic  29 , Vanessa Smith  30 , Carolina de Souza Müller  31 , Carlo Maurizio Montecucco  32 , Daniela Opris  33 , Francesca Ingegnoli  34 , Panayiotis G Vlachoyiannopoulos  35 , Bojana Stamenkovic  36 , Edoardo Rosato  37 , Stefan Heitmann  38 , Jörg H W Distler  39 , Thierry Zenone  40 , Matthias Seidel  41 , Alessandra Vacca  42 , Ellen De Langhe  43 , Srdan Novak  44 , Maurizio Cutolo  45 , Luc Mouthon  46 , Jörg Henes  47 , Carlo Chizzolini  48 , Carlos Alberto von Mühlen  49 , Kamal Solanki  50 , Simona Rednic  51 , Lisa Stamp  52 , Branimir Anic  53 , Vera Ortiz Santamaria  54 , Maria De Santis  55 , Sule Yavuz  56 , Walter Alberto Sifuentes-Giraldo  57 , Emmanuel Chatelus  58 , Jiri Stork  59 , Jacob van Laar  60 , Esthela Loyo  61 , Paloma García de la Peña Lefebvre  62 , Kilian Eyerich  63 , Vanesa Cosentino  64 , Juan Jose Alegre-Sancho  65 , Otylia Kowal-Bielecka  66 , Grégoire Rey  67 , Marco Matucci-Cerinic  68 , Yannick Allanore  1 ; EUSTAR group
Affiliations

Mapping and predicting mortality from systemic sclerosis

Muriel Elhai et al. Ann Rheum Dis. 2017 Nov.

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: cardiovascular disease; epidemiology; pulmonary fibrosis; systemic sclerosis.

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Conflict of interest statement

Competing interests: OD reports personal fees from 4D Science, grants and personal fees from Actelion, personal fees from Active Biotech, grants and personal fees from Bayer, personal fees from Biogen Idec, personal fees from BMS, grants and personal fees from Boehringer Ingelheim, personal fees from ChemomAb, personal fees from EpiPharm, personal fees from EspeRare Foundation, personal fees from Genentech/Roche, personal fees from GSK, personal fees from Inventiva, personal fees from Lilly, personal fees from Medac, personal fees from Mepha, personal fees from MedImmune, personal fees from Pharmacyclics, grants and personal fees from Pfizer, grants and personal fees from Sanofi, personal fees from Serodapharm, personal fees from Sinoxa, personal fees from AbbVie, personal fees from iQone Healthcare, outside the submitted work. In addition, OD has a patent mir-29 for the treatment of systemic sclerosis licensed. FI reports personal fees from AbbVie, personal fees from BMS, personal fees from MSD, personal fees from Novartis, outside the submitted work. JHWD reports personal fees from Actelion, grants and personal fees from Anamar, grants and personal fees from Bayer Pharma, grants and personal fees from Boehringer Ingelheim, grants from Celgene, personal fees from Galapagos, grants from GSK, grants and personal fees from Inventiva, personal fees from Pfizer, grants and personal fees from UCB, grants from Novartis, other from 4D Science, outside the submitted work. JvL reports personal fees from Pfizer, grants and personal fees from MSD, personal fees from Eli Lilly, personal fees from BMS, personal fees from Roche, outside the submitted work. Other coauthors have nothing to disclose.

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