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FRI0393 Predictive value of history of digital ulcers in a eustar cohort of patients with systemic sclerosis
  1. C. Mihai1,
  2. R. Landewé2,
  3. D. van der Heijde3,
  4. U. Walker4,
  5. P. I. Constantin1,
  6. R. M. Ionescu5,
  7. S. Rednic6,
  8. A. M. Gherghe1,
  9. R. Sfrent-Cornăteanu7,
  10. Y. Allanore8,
  11. J. Avouac8,
  12. L. Czirjak9,
  13. D. Farge-Bancel10,
  14. N. Damjanov11,
  15. O. Kowal-Bielecka12,
  16. F. van den Hoogen13,
  17. A. Tyndall14,
  18. M. Cutolo15,
  19. U. Müller-Ladner16,
  20. M. Matucci Cerinic17,
  21. EUSTAR
  1. 1Internal Medicine and Rheumatology, Cantacuzino Hospital, Carol Davila University, Bucharest, Romania
  2. 2Academic Medical Center, University of Amsterdam, Amsterdam
  3. 3Leiden University Medical Center, Leiden, Netherlands
  4. 4Felix-Platter Spital, Basel, Switzerland
  5. 5Internal Medicine and Rheumatology, Sf Maria Hospital, Carol Davila University, Bucharest
  6. 6Rheumatology Clinic, Iuliu Hatieganu University, Cluj-Napoca
  7. 7Immunology, Carol Davila University, Bucharest, Romania
  8. 8Rhumatologie A & INSERM U1016, Hopital Cochin, Université Paris Descartes, Paris, France
  9. 9Rheumatology, University of Pecs, Pecs, Hungary
  10. 10Internal Medicine and Vascular Pathology, Hopital St Louis, Université Denis Diderot, Paris, France
  11. 11Institute of Rheumatology, Belgrade University School of Medicine, Belgrade, Serbia
  12. 12Internal Medicine and Rheumatology, Medical University of Bialystok, Bialystok, Poland
  13. 13Rheumatology, Maartenskliniek, Nijmegen, Netherlands
  14. 14Rheumatology, Felix-Platter Spital, Basel, Switzerland
  15. 15Rheumatology, University of Genoa, Genoa, Italy
  16. 16Internal Medicine and Rheumatology, Justus-Liebig University, Giessen, Germany
  17. 17Rheumatology, Uniiversity of Florence, Florence, Italy

Abstract

Background Digital ulcers (DUs) have a prevalence of up to 36% in systemic sclerosis (SSc) patients and are a heavy burden, significantly reducing quality of life and function and leading to severe complications.

Objectives To evaluate in SSc patients the hypothesis that a history of DUs at presentation may be a predictor of vascular outcomes and of overall clinical worsening and death.

Methods All patients from the EUSTAR database (MEDS online), satisfying the 1980 ACR classification criteria for SSc, who had a follow-up of at least 3 years since inclusion or who have died, were included in the analysis. History of DUs at presentation as a predictor of disease progression or death was evaluated by univariate and multivariate Cox proportional hazard regression analysis.

Results 3207 patients matched the inclusion criteria (age at inclusion 54.6±13.6 years, disease duration since the onset of first non-Raynaud’s symptom: 8.1±7.7 years and from onset of Raynaud’s phenomenon (RP) 12.3±11.2 years, male sex 13.2%, 33.4% diffuse subset, 96.4% presenting RP). At presentation, 1092 patients had a history of DUs (34.1%). DU history at baseline was the strongest predictor for the presence of active DUs at prospective visits, with a hazard ratio (HR, 95% confidence interval) of 2.55 (2.02-3.20), p<0.001 when adjusting for age, gender and all parameters found significant in univariate analysis (elevated systolic pulmonary arterial pressure by ultrasound (sPAP-US), decreased carbon monoxyde lung diffusion (DLCO), anti-Scl70 antibodies). In univariate age-adjusted analysis, DUs were predictive of severe interstitial lung disease, defined as a forced vital capacity (FVC) of <50% predicted: HR 1.75 (1.59-2.65), p<0.01, and for severely decreased DLCO (<40% predicted), HR 1.47(1.22-1.76), p<0.001, but in multivariate analysis this association was not statistically significant anymore.

There were 453 deaths recorded. The mean±SD time to death since RP onset was 17.3±11.3 years and since first non-Raynaud symptom 8.9±8.6 years. DUs were found to be predictive for death (HR 1.50,(1.15-1.96), p<0.001) after adjusting for age, gender and all baseline parameters found to be significantly associated in the univariate analysis (disease subset, elevated sPAP-US, proteinuria, significant dyspnea, decreased FVC and DLCO, history of renal crisis and presence of anti-Scl70 antibodies).

Conclusions A history of DUs at presentation is a predictor for the presence of DUs at follow-up and forecasts early mortality in patients with SSc. As such, the presence of DUs in a patient with SSc predicts a poor outcome.

Disclosure of Interest: None Declared

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