Primary systemic sclerosis heart involvement: A systematic literature review and preliminary data-driven, consensus-based WSF/HFA definition

1*, 2, 3*, 1, 4, 5, 6, 7, 8, 9, 6, 10, 11, 12, 6, 13, 14, 15, 16, 17, 1, 17, 18, 13, 19, 20, 6, 21, 22, 23, 24, 9, 16, 25, 26, 27, 13, 28, 29, 30, 31, 6, 7, 32§, 1, 5, 33§

1Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Florence, Italy

2Centre for Musculoskeletal Research, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK

3NIHR Biomedical Research Centre, Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK

4Department Rheumatology, University of California, Los Angeles, Los Angeles, CA, USA

5Unit of Immunology, Rheumatology Allergy and Rare diseases, IRCSS San Raffaele Hospital, Vita Salute San Raffaele University, Milano, Italy

6Department of Cardiology, University Hospital Centre Bezanijska Kosa, Belgrade, Serbia

7School of Medicine, University of Belgrade, Belgrade, Serbia

8Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK

9Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy

10Rheumatology Department, Paris University, Cochin Hospital, Paris, France

11Division of Cardiovascular Sciences, University of Manchester, Manchester, UK

12Department of Rheumatology and Rehabilitation, Faculty of Medicine, Assuit University Hospital, Assuit, Arab Republic of Egypt

13Cardiology and Cardio-Immunology Outpatient Clinic, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy

14Department of Medicine, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA

15Department of Cardiology, Mitera General Hospital-Hygeia Group, Athens, Greece

16Department of Allergy and Rheumatology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan

17Onassis Cardiac Surgery Centre and Kapodistrian University of Athens, Athens, Attica, Greece

18Institute of Clinical Physiology, National Research Council, Pisa, Italy

19European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), Brussels, Belgium

20Department of Internal Medicine and Cardiology, Charité Campus Virchow-Klinikum, Charité – University Medicine Berlin, Berlin, Germany

21Institute for Pathology and Neuropathology, Cardiopathology, University of Tuebingen, Tuebingen, Germany

22Leeds Institute of Cardiovascular and Metabolic Medicine, University of Leeds, Leeds, UK

23Cardiology Clinical Academic Group, Institute of Molecular and Clinical Sciences, St George’s University of London, London, UK

24St George’s University Hospitals NHS Foundation Trust, London, UK

25Department Rheumatology, Royal Free Hospital, London, UK

26University of Michigan Scleroderma Program, Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA

27Translational Matrix Biology, Medical Faculty, University of Cologne, Cologne, Germany

28Haematology and Clinical Immunology Unit, University Hospital of Padua, Padua, Italy

29Federation of European Scleroderma Associations (FESCA), Brussels, Belgium

30Department of Medicine, Division of Rheumatology, University of Padua, Padova, Italy

31Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy

32Serbian Academy of Sciences and Arts, Belgrade, Serbia

33Department of Geriatric Medicine, Division of Rheumatology, Careggi University Hospital, Florence, Italy


*Equal contribution as first authors.

§Equal contribution as senior author.

Corresponding Author:

Cosimo Bruni, Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Via delle Oblate 4, 50141 Florence, Italy. Email: Maya H Buch, Centre for Musculoskeletal Research, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, AV Hill Building, Manchester M13 9LJ, UK. Email:

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