Cosimo Bruni1, Maya H Buch2,3, Daniel E Furst1,4, Giacomo De Luca5, Aleksandra Djokovic6,7, Raluca B Dumitru8, Alessandro Giollo9, Marija Polovina6, Alexia Steelandt10, Kostantinos Bratis11, Yossra Atef Suliman12, Ivan Milinkovic6, Anna Baritussio13, Ghadeer Hasan14, Anastasia Xintarakou15, Yohei Isomura16, George Markousis-Mavrogenis17, Lorenzo Tofani1, Sophie Mavrogeni17, Luna Gargani18, Alida Lp Caforio13,19, Carsten Tschöpe20, Arsen Ristic6, Karin Klingel21, Sven Plein22, Elijah R Behr23,24, Yannick Allanore9, Masataka Kuwana16, Christopher P Denton25, Dinesh Khanna26, Thomas Krieg27, Renzo Marcolongo13,28, Ilaria Galetti29, Elisabetta Zanatta30, Francesco Tona31, Petar Seferovic6,7,32, Marco Matucci-Cerinic1,5,33. 1. Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Florence, Italy. 2. Centre for Musculoskeletal Research, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK. 3. NIHR Biomedical Research Centre, Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. 4. Department Rheumatology, University of California, Los Angeles, Los Angeles, CA, USA. 5. Unit of Immunology, Rheumatology Allergy and Rare diseases, IRCSS San Raffaele Hospital, Vita Salute San Raffaele University, Milano, Italy. 6. Department of Cardiology, University Hospital Centre Bezanijska Kosa, Belgrade, Serbia. 7. School of Medicine, University of Belgrade, Belgrade, Serbia. 8. Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. 9. Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy. 10. Rheumatology Department, Paris University, Cochin Hospital, Paris, France. 11. Division of Cardiovascular Sciences, University of Manchester, Manchester, UK. 12. Department of Rheumatology and Rehabilitation, Faculty of Medicine, Assuit University Hospital, Assuit, Arab Republic of Egypt. 13. Cardiology and Cardio-Immunology Outpatient Clinic, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy. 14. Department of Medicine, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA. 15. Department of Cardiology, Mitera General Hospital-Hygeia Group, Athens, Greece. 16. Department of Allergy and Rheumatology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan. 17. Onassis Cardiac Surgery Centre and Kapodistrian University of Athens, Athens, Attica, Greece. 18. Institute of Clinical Physiology, National Research Council, Pisa, Italy. 19. European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), Brussels, Belgium. 20. Department of Internal Medicine and Cardiology, Charité Campus Virchow-Klinikum, Charité - University Medicine Berlin, Berlin, Germany. 21. Institute for Pathology and Neuropathology, Cardiopathology, University of Tuebingen, Tuebingen, Germany. 22. Leeds Institute of Cardiovascular and Metabolic Medicine, University of Leeds, Leeds, UK. 23. Cardiology Clinical Academic Group, Institute of Molecular and Clinical Sciences, St George's University of London, London, UK. 24. St George's University Hospitals NHS Foundation Trust, London, UK. 25. Department Rheumatology, Royal Free Hospital, London, UK. 26. University of Michigan Scleroderma Program, Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA. 27. Translational Matrix Biology, Medical Faculty, University of Cologne, Cologne, Germany. 28. Haematology and Clinical Immunology Unit, University Hospital of Padua, Padua, Italy. 29. Federation of European Scleroderma Associations (FESCA), Brussels, Belgium. 30. Department of Medicine, Division of Rheumatology, University of Padua, Padova, Italy. 31. Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy. 32. Serbian Academy of Sciences and Arts, Belgrade, Serbia. 33. Department of Geriatric Medicine, Division of Rheumatology, Careggi University Hospital, Florence, Italy.
Abstract
Introduction: Primary heart involvement in systemic sclerosis may cause morpho-functional and electrical cardiac abnormalities and is a common cause of death. The absence of a clear definition of primary heart involvement in systemic sclerosis limits our understanding and ability to focus on clinical research. We aimed to create an expert consensus definition for primary heart involvement in systemic sclerosis. Methods: A systematic literature review of cardiac involvement and manifestations in systemic sclerosis was conducted to inform an international and multi-disciplinary task force. In addition, the nominal group technique was used to derive a definition that was then subject to voting. A total of 16 clinical cases were evaluated to test face validity, feasibility, reliability and criterion validity of the newly created definition. Results: In total, 171 publications met eligibility criteria. Using the nominal group technique, experts added their opinion, provided statements to consider and ranked them to create the consensus definition, which received 100% agreement on face validity. A median 60(5-300) seconds was taken for the feasibility on a single case. Inter-rater agreement was moderate (mKappa (95% CI) = 0.56 (0.46-1.00) for the first round and 0.55 (0.44-1.00) for the second round) and intra-rater agreement was good (mKappa (95% CI) = 0.77 (0.47-1.00)). Criterion validity showed a 78 (73-84)% correctness versus gold standard. Conclusion: A preliminary primary heart involvement in systemic sclerosis consensus-based definition was created and partially validated, for use in future clinical research.
Introduction: Primary heart involvement in systemic sclerosis may cause morpho-functional and electrical cardiac abnormalities and is a common cause of death. The absence of a clear definition of primary heart involvement in systemic sclerosis limits our understanding and ability to focus on clinical research. We aimed to create an expert consensus definition for primary heart involvement in systemic sclerosis. Methods: A systematic literature review of cardiac involvement and manifestations in systemic sclerosis was conducted to inform an international and multi-disciplinary task force. In addition, the nominal group technique was used to derive a definition that was then subject to voting. A total of 16 clinical cases were evaluated to test face validity, feasibility, reliability and criterion validity of the newly created definition. Results: In total, 171 publications met eligibility criteria. Using the nominal group technique, experts added their opinion, provided statements to consider and ranked them to create the consensus definition, which received 100% agreement on face validity. A median 60(5-300) seconds was taken for the feasibility on a single case. Inter-rater agreement was moderate (mKappa (95% CI) = 0.56 (0.46-1.00) for the first round and 0.55 (0.44-1.00) for the second round) and intra-rater agreement was good (mKappa (95% CI) = 0.77 (0.47-1.00)). Criterion validity showed a 78 (73-84)% correctness versus gold standard. Conclusion: A preliminary primary heart involvement in systemic sclerosis consensus-based definition was created and partially validated, for use in future clinical research.
Authors: Frank van den Hoogen; Dinesh Khanna; Jaap Fransen; Sindhu R Johnson; Murray Baron; Alan Tyndall; Marco Matucci-Cerinic; Raymond P Naden; Thomas A Medsger; Patricia E Carreira; Gabriela Riemekasten; Philip J Clements; Christopher P Denton; Oliver Distler; Yannick Allanore; Daniel E Furst; Armando Gabrielli; Maureen D Mayes; Jacob M van Laar; James R Seibold; Laszlo Czirjak; Virginia D Steen; Murat Inanc; Otylia Kowal-Bielecka; Ulf Müller-Ladner; Gabriele Valentini; Douglas J Veale; Madelon C Vonk; Ulrich A Walker; Lorinda Chung; David H Collier; Mary Ellen Csuka; Barri J Fessler; Serena Guiducci; Ariane Herrick; Vivien M Hsu; Sergio Jimenez; Bashar Kahaleh; Peter A Merkel; Stanislav Sierakowski; Richard M Silver; Robert W Simms; John Varga; Janet E Pope Journal: Arthritis Rheum Date: 2013-10-03
Authors: Lesley-Anne Bissell; Marina Anderson; Malcolm Burgess; Kuntal Chakravarty; Gerry Coghlan; Raluca B Dumitru; Lee Graham; Voon Ong; John D Pauling; Sven Plein; Dominik Schlosshan; Peter Woolfson; Maya H Buch Journal: Rheumatology (Oxford) Date: 2017-06-01 Impact factor: 7.580
Authors: Maarten Boers; John R Kirwan; George Wells; Dorcas Beaton; Laure Gossec; Maria-Antonietta d'Agostino; Philip G Conaghan; Clifton O Bingham; Peter Brooks; Robert Landewé; Lyn March; Lee S Simon; Jasvinder A Singh; Vibeke Strand; Peter Tugwell Journal: J Clin Epidemiol Date: 2014-02-28 Impact factor: 6.437
Authors: Murray Baron; Bashar Kahaleh; Elana J Bernstein; Lorinda Chung; Philip J Clements; Christopher Denton; Robyn T Domsic; Nava Ferdowsi; Ivan Foeldvari; Tracy Frech; Jessica K Gordon; Marie Hudson; Sindhu R Johnson; Dinesh Khanna; Zsuzsannah McMahan; Peter A Merkel; Sonali Narain; Mandana Nikpour; John D Pauling; Laura Ross; Antonia Maria Valenzuela Vergara; Alessandra Vacca Journal: J Scleroderma Relat Disord Date: 2018-07-18
Authors: Y A Suliman; C Bruni; S R Johnson; E Praino; M Alemam; N Borazan; L Cometi; B Meyers; D Khanna; Y Allanore; M Baron; T Krieg; A Herrick; A Afonso; O Distler; S Kafaja; C P Denton; M Matucci-Cerinic; D E Furst Journal: J Scleroderma Relat Disord Date: 2017-05-19