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Clinical prediction of 5-year survival in systemic sclerosis: validation of a simple prognostic model in EUSTAR centres
  1. J Fransen1,
  2. D Popa-Diaconu1,
  3. R Hesselstrand2,
  4. P Carreira3,
  5. G Valentini4,
  6. L Beretta5,6,
  7. P Airo7,
  8. M Inanc8,
  9. S Ullman9,
  10. A Balbir-Gurman10,
  11. S Sierakowski11,
  12. Y Allanore12,
  13. L Czirjak13,
  14. V Riccieri14,
  15. R Giacomelli15,
  16. A Gabrielli16,
  17. G Riemekasten17,
  18. M Matucci-Cerinic18,
  19. D Farge19,
  20. N Hunzelmann20,
  21. F H J Van den Hoogen21,
  22. M C Vonk1
  1. 1Department of Rheumatology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands
  2. 2Department of Rheumatology, Lund University Hospital, Lund, Sweden
  3. 3Department of Rheumatology, Hospital de 12 octobre, Madrid, Spain
  4. 4Rheumatology Unit, Second University of Naples, Naples, Italy
  5. 5Referral Centers for Systemic Autoimmune Diseases, University of Milan, Italy
  6. 6Fondazione IRCCS Ospedale Maggiore Policlinico, Milan, Italy
  7. 7Department of Rheumatology, University of Brescia, Brescia, Italy
  8. 8Division of Rheumatology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey
  9. 9Department of Dermatology, Copenhagen University Hospital, Copenhagen, Denmark
  10. 10B Shine Rheumatology Unit, Rambam Medical Centre, Haifa, Israel
  11. 11Department of Rheumatology and Internal Diseases, Medical Academic Hospital, Bialystok, Poland
  12. 12Université Paris Descartes, Rhumatologie A, Hôpital Cochin, APHP, Paris, France
  13. 13Department of Immunology and Rheumatology, University of Pécs, Pécs, Hungary
  14. 14Division of Rheumatology, Department of Clinical Medicine and Therapy, University of Rome ‘Sapienza’, Rome, Italy
  15. 15Department of Internal Medicine and Public Health, University of L'Aquila, L'Aquila, Italy
  16. 16Department of Medical Science and Surgery, Università Politecnica delle Marche and Ospedali Riuniti, Ancona, Italy
  17. 17Department of Rheumatology and Clinical Immunology, Charité, Humboldt-University, Berlin, Germany
  18. 18Division of Medicine and Rheumatology, University of Florence, Florence, Italy
  19. 19Department of Internal Medicine, Hôpital Saint-Louis, Paris, France
  20. 20Department of Dermatology, University of Cologne, Cologne, Germany
  21. 21 Department of Rheumatology, Maartenskliniek, Nijmegen, The Netherlands
  1. Correspondence to J Fransen, Department of Rheumatology (470), Radboud University Nijmegen Medical Center, P O Box 9101, 6500 HB, Nijmegen, The Netherlands; j.fransen{at}


Objective Systemic sclerosis (SSc) is associated with a significant reduction in life expectancy. A simple prognostic model to predict 5-year survival in SSc was developed in 1999 in 280 patients, but it has not been validated in other patients. The predictions of a prognostic model are usually less accurate in other patients, especially from other centres or countries. A study was undertaken to validate the prognostic model to predict 5-year survival in SSc in other centres throughout Europe.

Methods A European multicentre cohort of patients with SSc diagnosed before 2002 was established. Patients with SSc according to the preliminary American College of Rheumatology classification criteria were eligible for the study when they were followed for at least 5 years or shorter if they died. The primary outcome was 5-year survival after diagnosis of SSc. The predefined prognostic model uses the following baseline variables: age, gender, presence of urine protein, erythrocyte sedimentation rate (ESR) and carbon monoxide diffusing capacity (DLCO).

Results Data were available for 1049 patients, 119 (11%) of whom died within 5 years after diagnosis. Of the patients, 85% were female, the mean (SD) age at diagnosis was 50 (14) years and 30% were classified as having diffuse cutaneous SSc. The prognostic model with age (OR 1.03), male gender (OR 1.93), urine protein (OR 2.29), elevated ESR (1.89) and low DLCO (OR 1.94) had an area under the receiver operating characteristic curve of 0.78. Death occurred in 12 (2.2%) of 509 patients with no risk factors, 45 (13%) of 349 patients with one risk factor, 55 (33%) of 168 patients with two risk factors and 7 (30%) of 23 patients with three risk factors.

Conclusion A simple prognostic model using three disease factors to predict 5-year survival at diagnosis in SSc showed reasonable performance upon validation in a European multicentre study.

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  • Funding This study was endorsed and has been supported by an epidemiological grant from the European League against Rheumatism - Scleroderma Trials and Research group (EUSTAR).

  • Competing interests None.

  • Ethics approval Because this is a multicentre study, each institution obtained ethical approval from its own ethics committee.

  • Provenance and peer review Not commissioned; externally peer reviewed.