Causes and risk factors for death in systemic sclerosis: A study from the EULAR Scleroderma Trials and Research (EUSTAR) database

Anthony J. Tyndall, Bettina Bannert, Madelon Vonk, Paolo Airò, Franco Cozzi, Patricia E. Carreira, Dominique Farge Bancel, Yannick Allanore, Ulf Müller-Ladner, Oliver Distler, Florenzo Iannone, Raffaele Pellerito, Margarita Pileckyte, Irene Miniati, Lidia Ananieva, Alexandra Balbir Gurman, Nemanja Damjanov, Adelheid Mueller, Gabriele Valentini, Gabriela RiemekastenMohammed Tikly, Laura Hummers, Maria J.S. Henriques, Paola Caramaschi, Agneta Scheja, Blaz Rozman, Evelien Ton, Gábor Kumánovics, Bernard Coleiro, Eva Feierl, Gabriella Szucs, Carlos Alberto Von Mühlen, Valeria Riccieri, Srdan Novak, Carlo Chizzolini, Anna Kotulska, Christopher Denton, Paulo C. Coelho, Ina Kötter, Ismail Simsek, Paloma García De La Pena Lefebvre, Eric Hachulla, James R. Seibold, Simona Rednic, Jiří Štork, Jadranka Morovic-Vergles, Ulrich A. Walker

634 Citations (Scopus)


Objectives: To determine the causes and predictors of mortality in systemic sclerosis (SSc). Methods: Patients with SSc (n=5860) fulfilling the American College of Rheumatology criteria and prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) cohort were analysed. EUSTAR centres completed a structured questionnaire on cause of death and comorbidities. Kaplan-Meier and Cox proportional hazards models were used to analyse survival in SSc subgroups and to identify predictors of mortality. Results: Questionnaires were obtained on 234 of 284 fatalities. 55% of deaths were attributed directly to SSc and 41% to non-SSc causes; in 4% the cause of death was not assigned. Of the SSc-related deaths, 35% were attributed to pulmonary fibrosis, 26% to pulmonary arterial hypertension (PAH) and 26% to cardiac causes (mainly heart failure and arrhythmias). Among the non-SSc-related causes, infections (33%) and malignancies (31%) were followed by cardiovascular causes (29%). Of the non-SSc-related fatalities, 25% died of causes in which SSc-related complications may have participated (pneumonia, sepsis and gastrointestinal haemorrhage). Independent risk factors for mortality and their HR were: proteinuria (HR 3.34), the presence of PAH based on echocardiography (HR 2.02), pulmonary restriction (forced vital capacity below 80% of normal, HR 1.64), dyspnoea above New York Heart Association class II (HR 1.61), diffusing capacity of the lung (HR 1.20 per 10% decrease), patient age at onset of Raynaud's phenomenon (HR 1.30 per 10 years) and the modified Rodnan skin score (HR 1.20 per 10 score points). Conclusion: Disease-related causes, in particular pulmonary fibrosis, PAH and cardiac causes, accounted for the majority of deaths in SSc.

Original languageEnglish
JournalAnnals of the Rheumatic Diseases
Issue number10
Pages (from-to)1809-1815
Number of pages7
Publication statusPublished - 10.2010

Research Areas and Centers

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


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