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Vasodilators and low-dose acetylsalicylic acid are associated with a lower incidence of distinct primary myocardial disease manifestations in systemic sclerosis: results of the DeSScipher inception cohort study
  1. Gabriele Valentini1,
  2. Dörte Huscher2,3,
  3. Antonella Riccardi1,
  4. Serena Fasano1,
  5. Rosaria Irace1,
  6. Valentina Messiniti1,
  7. Marco Matucci-Cerinic4,
  8. Serena Guiducci4,
  9. Oliver Distler5,
  10. Britta Maurer5,
  11. Jérôme Avouac6,
  12. Ingo H Tarner7,
  13. Marc Frerix7,
  14. Gabriela Riemekasten8,
  15. Elise Siegert9,
  16. László Czirják10,
  17. Veronika Lóránd10,
  18. Christopher P Denton11,
  19. Svetlana Nihtyanova11,
  20. Ulrich A Walker12,
  21. Veronika K Jaeger12,
  22. Francesco Del Galdo13,
  23. Giuseppina Abignano13,14,
  24. Lidia P Ananieva15,
  25. Ana Maria Gherghe16,
  26. Carina Mihai16,
  27. Joerg Christoph Henes17,
  28. Tim Schmeiser18,
  29. Alessandra Vacca19,
  30. Sergey Moiseev20,
  31. Ivan Foeldvari21,
  32. Armando Gabrielli22,
  33. Brigitte Krummel-Lorenz23,
  34. Simona Rednic24,
  35. Yannick Allanore6,
  36. Ulf Müeller-Ladner7
  1. 1 Department of Precision Medicine, Section of Rheumatology, University of Campania Luigi Vanvitelli, Naples, Italy
  2. 2 Institute of Biostatistics and Clinical Epidemiology, Charité Universitätsmedizin Berlin, Berlin, Germany
  3. 3 Berlin Institute of Health, Berlin, Germany
  4. 4 Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Florence, Italy
  5. 5 Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland
  6. 6 Department of Rheumatology, Cochin Hospital, University of Paris Descartes, Paris, France
  7. 7 Department of Rheumatology and Clinical Immunology, Justus Liebig Universitat Giessen, Bad Nauheim, Germany
  8. 8 Department of Rheumatology, Universitatsklinikum Schleswig Holstein—Campus Lubeck, Lübeck, Germany
  9. 9 Department of Rheumatology and Clinical Immunology, Charité - Universitäetsmedizin Berlin, Berlin, Germany
  10. 10 Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary
  11. 11 Department of Rheumatology, University College London, Royal Free Hospital, London, United Kingdom
  12. 12 Department of Rheumatology, University of Basel, Basel, Switzerland
  13. 13 Leeds Biomedical Research Centre and Leeds Institute of Rheumatic and Musculoskeletal Medicine, Leeds, United Kingdom
  14. 14 Rheumatology Institute of Lucania, San Carlo Hospital of Potenza and Madonna delle Grazie Hospital of Matera, Potenza, Italy
  15. 15 Institute of Rheumatology, Russian Academy of Medical Science, Moscow, Russian Federation
  16. 16 Internal Medicine and Rheumatology Department, Cantacuzino Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania
  17. 17 Department of Internal Medicine II, University Hospitals Tübingen, Tübingen, Germany
  18. 18 Department of Rheumatology and Immunology, Saint Josef Hospital, Wuppertal, Germany
  19. 19 Rheumatology Unit, University of Cagliari, Cagliari, Italy
  20. 20 Sechenov First Moscow State Medical University and Lomonosov Moscow State University, Moscow, Russia
  21. 21 Klinikum Eilbek, Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany
  22. 22 Clinical Medicine, Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona, Italy
  23. 23 Endokrinologikum Frankfurt, Frankfurt, Germany
  24. 24 Department of Rheumatology, University of Medicine and Pharmacy ‘luliu Hatieganu’ Cluj, Cluj-Napoca, Romania
  1. Correspondence to Professor Gabriele Valentini, Department of Precision Medicine, Section of Rheumatology, University of Campania Luigi Vanvitelli, Naples 80131, Italy; gabriele.valentini{at}


Objectives To investigate the influence of vasodilator drugs on the occurrence of features depending on myocardial ischaemia/fibrosis (ventricular arrhythmias, Q waves, cardiac blocks, pacemaker implantation, left ventricular ejection fraction (LVEF) <55%, and/or congestive heart failure and sudden cardiac death) in systemic sclerosis (SSc).

Methods 601 patients with SSc were enrolled from 1 December 2012 to 30 November 2015 and had a second visit 0.5–4 years apart. 153 received no vasodilators; 448 received vasodilator therapy (ie, calcium channel blockers and/or ACE inhibitors or angiotensin II receptor blockers or combinations of them), 89 of them being also treated with either endothelin receptor antagonists or PDE5 inhibitors or prostanoids. Associations between the occurrence of myocardial disease manifestations and any demographic, disease and therapeutic aspect were investigated by Cox regression analysis. A Cox frailty survival model with centre of enrolment as random effect was performed.

Results During 914 follow-up patient-years, 12 ventricular arrhythmias, 5 Q waves, 40 cardiac blocks, 6 pacemaker implantations and 19 reduced LVEF and/or congestive heart failure (CHF) occurred. In multivariate Cox regression analysis, vasodilator therapy was associated with a lower incidence of ventricular arrhythmias (p=0.03); low-dose acetylsalicylic acid (ASA) with a lower incidence of cardiac blocks and/or Q waves and/or pacemaker implantation (p=0.02); active disease with a higher incidence of LVEF <55% and/or CHF and cardiac blocks and/or Q waves and/or pacemaker implantation (p=0.05).

Conclusions The present study might suggest a preventative effect on the occurrence of distinct myocardial manifestations by vasodilator therapy and low-dose ASA.

  • primary myocardial disease in scleroderma
  • preventative role of vasodilator therapy

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  • Handling editor Josef S Smolen

  • Contributors Study conception and design: GV, UM-L, CPD, FDG, GR, LC, MM-C, OD, UAW, YA. Acquisition of data: AR, SF, RI, VM, SG, BM, JA, IHT, MF, ES, VL, SN, VKJ, GA, LPA, AMG, CM, JCH, TS, AV, SM, IF, AG, BK-L, SR. Analysis and interpretation of data: GV, DH, AR, SF. Revising the article: GV, BM, IHT, LC, CPD, UAW, YA, UM-L.

  • Funding Funded by the European Community FP7 programme (DeSScipher FP7-HEALTH no. 305495) and European Scleroderma Trials and Research group (EUSTAR)

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Ethics approval All contributing EUSTAR centres have obtained approval from their respective local ethics committee for including patient data in the EUSTAR database.

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

  • Data availability statement Data are available on reasonable request.

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