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Extended report
A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: a EUSTAR prospective study
  1. Muriel Elhai1,
  2. Jérôme Avouac1,
  3. Ulrich A Walker2,
  4. Marco Matucci-Cerinic3,
  5. Gabriela Riemekasten4,
  6. Paolo Airò5,
  7. Eric Hachulla6,
  8. Gabriele Valentini7,
  9. Patricia E Carreira8,
  10. Franco Cozzi9,
  11. Alexandra Balbir Gurman10,
  12. Yolanda Braun-Moscovici10,
  13. Nemanja Damjanov11,
  14. Lidia P Ananieva12,
  15. Raffaella Scorza13,
  16. Sergio Jimenez14,
  17. Joanna Busquets14,
  18. Mengtao Li15,
  19. Ulf Müller-Ladner16,
  20. André Kahan1,
  21. Oliver Distler17,
  22. Yannick Allanore1,
  23. EUSTAR co-authors
    1. 1Department of Rheumatology A, Paris Descartes University, Cochin Hospital, Paris, France
    2. 2Department of Rheumatology, Basel University, Unispital Basel, Basel, Switzerland
    3. 3Department of Experimental and Clinical Medicine, Section of Internal Medicine and Division of Rheumatology, Azienda Ospedaliero-Universitaria Careggi (AOUC), University of Florence, Florence, Italy
    4. 4Department of Rheumatology, Charitè University Hospital, Berlin, German Rheumatism Research Centre Berlin (DRFZ), a Leibniz institute, Berlin, Germany
    5. 5UO Reumatologia ed Immunologia Clinica Spedali Civili Brescia, Brescia, Italy
    6. 6Department of Internal Medicine, Hôpital Claude Huriez, University Lille Nord-de-France, Lille cedex, France
    7. 7Department of Clinical and Experimental Medicine “F-Magrassi” II, Naples, Italy
    8. 8Servicio de Reumatologia, Hospital Universitario 12 de Octubre, Madrid, Spain
    9. 9Rheumatology Unit, Department of Medicine—DIMED, University of Padova, Padova, Italy
    10. 10B. Shine Department of Rheumatology, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion—Institute of Technology, Haifa, Israel
    11. 11Institute of Rheumatology, University of Belgrade Medical School, Belgrade, Serbia
    12. 12Institute of Rheumatology, Russian Academy of Medical Science, Moscow, Russia
    13. 13U.O. Immunologia Clinica—Centro di Riferimento per le Malattie Autoimmuni Sistemiche, Milano, Italy
    14. 14Scleroderma Center of Thomas Jefferson University, Philadelphia, Pennsylvania, USA
    15. 15Department of Rheumatology, Peking Union Medical College Hospital (West Campus), Chinese Academy of Medical Sciences, Beijing, China
    16. 16Department of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Kerckhoff Clinic, Bad Nauheim, Germany
    17. 17Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland
    1. Correspondence to Dr Yannick Allanore, Paris Descartes University, Rheumatology A Department, Cochin Hospital, 27 rue du Faubourg Saint Jacques, Paris 75014, France; yannick.allanore{at}


    Objectives In agreement with other autoimmune diseases, systemic sclerosis (SSc) is associated with a strong sex bias. However, unlike lupus, the effects of sex on disease phenotype and prognosis are poorly known. Therefore, we aimed to determine sex effects on outcomes.

    Method We performed a prospective observational study using the latest 2013 data extract from the EULAR scleroderma trials and research (EUSTAR) cohort. We looked at (i) sex influence on disease characteristics at baseline and (ii) then focused on patients with at least 2 years of follow-up to estimate the effects of sex on disease progression and survival.

    Results 9182 patients with SSc were available (1321 men) for the baseline analyses. In multivariate analysis, male sex was independently associated with a higher risk of diffuse cutaneous subtype (OR: 1.68, (1.45 to 1.94); p<0.001), a higher frequency of digital ulcers (OR: 1.28 (1.11 to 1.47); p<0.001) and pulmonary hypertension (OR: 3.01 (1.47 to 6.20); p<0.003). In the longitudinal analysis (n=4499), after a mean follow-up of 4.9 (±2.7) years, male sex was predictive of new onset of pulmonary hypertension (HR: 2.66 (1.32 to 5.36); p=0.006) and heart failure (HR: 2.22 (1.06 to 4.63); p=0.035). 908 deaths were recorded, male sex predicted deaths of all origins (HR: 1.48 (1.19 to 1.84); p<0.001), but did not significantly account for SSc-related deaths.

    Conclusions Although more common in women, SSc appears as strikingly more severe in men. Our results obtained through the largest worldwide database demonstrate a higher risk of severe cardiovascular involvement in men. These results raise the point of including sex in the management and the decision-making process.

    • Autoimmune Diseases
    • Epidemiology
    • Systemic Sclerosis

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