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Joint and tendon involvement predict disease progression in systemic sclerosis: a EUSTAR prospective study
  1. Jérôme Avouac1,
  2. Ulrich A Walker2,
  3. Eric Hachulla3,
  4. Gabriela Riemekasten4,
  5. Giovanna Cuomo5,
  6. Patricia E Carreira6,
  7. Paola Caramaschi7,
  8. Lidia P Ananieva8,
  9. Marco Matucci-Cerinic9,
  10. Laszlo Czirjak10,
  11. Christopher Denton11,
  12. Ulf Müller Ladner12,
  13. Yannick Allanore1,
  14. the EUSTAR collaborators*
    1. 1Rheumatology A Department, Paris Descartes University, Cochin Hospital, Paris, France
    2. 2Department of Rheumatology, Basel University, Basel, Switzerland
    3. 3Department of Internal Medicine, Université Lille Nord de France, IMPRT IFR 114, Hôpital Claude-Huriez, and Centre Hospitalier Régional Universitaire de Lille, Lille, France
    4. 4Department of Rheumatology, Clinical Immunology, German Rheumatism Research Centre, a Leibniz institute, Charité-University Medicine, Berlin, Germany
    5. 5Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples, Naples, Italy
    6. 6Rheumatology Department, Hospital Universitario 12 de Octubre, Madrid, Spain
    7. 7Rheumatology Unit, Azienda Ospedaliera Universitaria Integrata, Verona, Italia
    8. 8Institute of Rheumatology, Russian Academy of Medical Science, Moscow, Russia
    9. 9Section of Rheumatology, Department of Medicine, University of Florence, Florence, Italy
    10. 10Department of Rheumatology and Immunology, University of Pecs, Pecs, Hungary
    11. 11Centre for Rheumatology, Royal Free and University College London Medical School, London, UK
    12. 12Department of Internal Medicine and Rheumatology, Justus-Liebig-University of Giessen, Kerckhoff-Klinik, Bad Nauheim, Germany
    1. Correspondence to Dr Jérôme Avouac, Rheumatology A Department, Paris Descartes University, Cochin Hospital, 27 rue du Faubourg Saint Jacques, Paris 75014, France; jerome.avouac{at}


    Objective To determine whether joint synovitis and tendon friction rubs (TFRs) can predict the progression of systemic sclerosis (SSc) over time.

    Patients and methods We performed a prospective cohort study that included 1301 patients with SSc from the EUSTAR database with disease duration ≤3 years at inclusion and with a follow-up of at least 2 years. Presence or absence at clinical examination of synovitis and TFRs was extracted at baseline. Outcomes were skin, cardiovascular, renal and lung progression. Overall disease progression was defined according to the occurrence of at least one organ progression.

    Results Joint synovitis (HR: 1.26, 95% CI 1.01 to 1.59) and TFRs (HR: 1.32, 95% CI 1.03 to 1.70) were independently predictive of overall disease progression, as were also the diffuse cutaneous subset (HR: 1.30, 95% CI 1.05 to 1.61) and positive antitopoisomerase-I antibodies (HR: 1.25, 95% CI 1.02 to 1.53). Regarding skin progression, joint synovitis (HR: 1.67, 95% CI 1.06 to 2.64) and TFRs (HR: 1.69, 95% CI 1.02 to 2.77) were also independently predictive of worsening of the modified Rodnan skin score. For cardiovascular progression, joint synovitis was predictive of the occurrence of new digital ulcer(s) (HR: 1.45, 95% CI 1.08 to 1.96) and decreased left ventricular ejection fraction (HR: 2.20, 95% CI 1.06 to 4.57); TFRs were confirmed to be an independent predictor of scleroderma renal crisis (HR: 2.33, 95% CI 1.03 to 6.19).

    Conclusions Joint synovitis and TFRs are independent predictive factors for disease progression in patients with early SSc. These easily detected clinical markers may be useful for the risk stratification of patients with SSc.

    • Systemic Sclerosis
    • Synovitis
    • Outcomes research

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