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

Research output: Contribution to journalArticle


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.


  • Anthony J. Tyndall
  • Bettina Bannert
  • Madelon Vonk
  • Paolo Airo
  • Franco Cozzi
  • Patricia E. Carreira
  • Dominique Farge Bancel
  • Yannick Allanore
  • Ulf Mueller-Ladner
  • Oliver Distler
  • Florenzo Iannone
  • Raffaele Pellerito
  • Margarita Pileckyte
  • Irene Miniati
  • Lidia Ananieva
  • Alexandra Balbir Gurman
  • Nemanja Damjanov
  • Adelheid Mueller
  • Gabriele Valentini
  • Gabriela Riemekasten
  • And 27 others
  • Mohammed Tikly
  • Laura Hummers
  • Maria J. S. Henriques
  • Paola Caramaschi
  • Agneta Scheja
  • Blaz Rozman
  • Evelien Ton
  • Gabor Kumanovics
  • Bernard Coleiro
  • Eva Feierl
  • Gabriella Szucs
  • Carlos Alberto Von Muehlen
  • Valeria Riccieri
  • Srdan Novak
  • Carlo Chizzolini
  • Anna Kotulska
  • Christopher Denton
  • Paulo C. Coelho
  • Ina Koetter
  • Ismail Simsek
  • Paloma Garcia de la Pena Lefebvre
  • Eric Hachulla
  • James R. Seibold
  • Simona Rednic
  • Jiri Stork
  • Jadranka Morovic-Vergles
  • Ulrich A. Walker
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Rheumatology and Autoimmunity
Original languageEnglish
Pages (from-to)1809-1815
JournalAnnals of the Rheumatic Diseases
Issue number10
Publication statusPublished - 2010
Publication categoryResearch