Mapping and predicting mortality from systemic sclerosis

Research output: Contribution to journalArticle


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.


  • EUSTAR group
External organisations
  • Technion - Israel Institute of Technology
  • 12 de Octubre University Hospital
  • University of Padova
  • Copenhagen University Hospital
  • University of Pécs
  • Justus Liebig University Giessen
  • University of Verona
  • University Hospital of Zurich
  • University of Bari Aldo Moro
  • Charles University in Prague
  • Marche Polytechnic University
  • University of Belgrade
  • Sapienza University of Rome
  • Carol Davila University of Medicine and Pharmacy
  • University Hospital Basel
  • University Hospital of Cologne
  • Clinical Hospital Split
  • Ghent University
  • Federal University of Paraná
  • Policlinico San Matteo Pavia Fondazione
  • University of Milan
  • National and Kapodistrian University of Athens
  • University Hospital Erlangen
  • Centre Hospitalier de Valence
  • University Hospital Bonn
  • University Hospitals Leuven
  • University of Genoa
  • Cochin Hospital
  • University Hospital of Tubingen
  • Geneva University Hospital
  • University of Paris 13
  • University of Lille
  • University of Lübeck
  • Spedali Civili Di Brescia
  • University of Campania Luigi Vanvitelli
  • Paris Descartes University
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Cardiac and Cardiovascular Systems


  • cardiovascular disease, epidemiology, pulmonary fibrosis, systemic sclerosis
Original languageEnglish
Pages (from-to)1897-1905
Number of pages9
JournalAnnals of the Rheumatic Diseases
Issue number11
Publication statusPublished - 2017 Nov 1
Publication categoryResearch