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Digital ulcers predict a worse disease course in patients with systemic sclerosis
  1. Carina Mihai1,
  2. Robert Landewé2,
  3. Désirée van der Heijde3,
  4. Ulrich A Walker4,
  5. Paul I Constantin1,
  6. Ana Maria Gherghe1,
  7. Ruxandra Ionescu5,
  8. Simona Rednic6,
  9. Yannick Allanore7,
  10. Jérôme Avouac7,
  11. László Czirják8,
  12. Eric Hachulla9,
  13. Gabriela Riemekasten10,
  14. Franco Cozzi11,
  15. Paolo Airò12,
  16. Maurizio Cutolo13,
  17. Ulf Mueller-Ladner14,
  18. Marco Matucci-Cerinic15,
  19. EUSTAR co-authors
    1. 1Department of Internal Medicine and Rheumatology, Cantacuzino Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania
    2. 2Amsterdam Rheumatology Center/University of Amsterdam & Atrium Medical Center, Heerlen, The Netherlands
    3. 3Leiden University Medical Center, Leiden, The Netherlands
    4. 4Basel University Hospital, Basel, Switzerland
    5. 5Department of Internal Medicine and Rheumatology, Sf. Maria Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania
    6. 6Rheumatology Clinic, Iuliu Haţieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania
    7. 7Rheumatology A Department, Paris Descartes University, Cochin Hospital, Paris, France
    8. 8Department of Rheumatology and Immunology, University of Pecs, Pecs, Hungary
    9. 9Internal Medicine Department, Claude Huriez Hospital, Lille, France
    10. 10Department of Rheumatology, Charité University Hospital, Berlin, Germany
    11. 11Cattedra di Reumatologia, Dip. Medicina Clinica e Sperimentale, Policlinico, University of Padova, Padova, Italy
    12. 12Spedali Civili di Brescia, Rheumatology and Clinical Immunology Service, Brescia, Italy
    13. 13University of Genova, Research Laboratory and Academic Clinical Unit of Rheumatology, Genova, Italy
    14. 14Department of Rheumatology and Clinical Immunology, Giessen University, Bad Nauheim, Germany
    15. 15Division of Rheumatology AOUC & Department of Experimental and Clinical Medicine, Department of Biomedicine, University of Florence, Florence, Italy
    1. Correspondence to Dr Carina Mihai, Department of Internal Medicine and Rheumatology, Cantacuzino Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Ion Movilă str. 5-7, Bucharest 020475, Romania; carinamihai{at}


    Objective Systemic sclerosis (SSc) is a systemic autoimmune disease with high morbidity and significant mortality. There is a great need of predictors that would allow risk stratification of patients with SSc and ultimately initiation of treatment early enough to ensure optimal clinical results. In this study, we evaluated whether a history of digital ulcers (HDU) at presentation may be a predictor of vascular outcomes and of overall clinical worsening and death in patients with SSc.

    Methods Patients from the EULAR Scleroderma Trials and Research (EUSTAR) database, satisfying at inclusion the 1980 American College of Rheumatology classification criteria for SSc, who had a follow-up of at least 3 years since baseline or who have died, were included in the analysis. HDU at presentation as a predictor of disease worsening or death was evaluated by Cox proportional hazards regression analysis.

    Results 3196 patients matched the inclusion criteria (male sex 13.2%, 33.4% diffuse subset). At presentation, 1092/3196 patients had an HDU (34.1%). In multivariable analysis adjusting for age, gender and all parameters considered potentially significant, HDU was predictive for the presence of active digital ulcers (DUs) at prospective visits (HR (95% CI)): 2.41 (1.91 to 3.03), p<0.001, for an elevated systolic pulmonary arterial pressure on heart ultrasound (US-PAPs):1.36 (1.03 to 1.80), p=0.032, for any cardiovascular event (new DUs, elevated US-PAPs or LV failure): 3.56 (2.26 to 5.62), p<0.001, and for death (1.53 (1.16 to 2.02), p=0.003).

    Conclusions In patients with SSc, HDU at presentation predicts the occurrence of DUs at follow-up and is associated with cardiovascular worsening and decreased survival.

    • Systemic Sclerosis
    • Cardiovascular Disease
    • Epidemiology

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