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Primary systemic sclerosis heart involvement: A systematic literature review and preliminary data-driven, consensus-based WSF/HFA definition.
Bruni, Cosimo; Buch, Maya H; Furst, Daniel E; De Luca, Giacomo; Djokovic, Aleksandra; Dumitru, Raluca B; Giollo, Alessandro; Polovina, Marija; Steelandt, Alexia; Bratis, Kostantinos; Suliman, Yossra Atef; Milinkovic, Ivan; Baritussio, Anna; Hasan, Ghadeer; Xintarakou, Anastasia; Isomura, Yohei; Markousis-Mavrogenis, George; Tofani, Lorenzo; Mavrogeni, Sophie; Gargani, Luna; Caforio, Alida Lp; Tschöpe, Carsten; Ristic, Arsen; Klingel, Karin; Plein, Sven; Behr, Elijah R; Allanore, Yannick; Kuwana, Masataka; Denton, Christopher P; Khanna, Dinesh; Krieg, Thomas; Marcolongo, Renzo; Galetti, Ilaria; Zanatta, Elisabetta; Tona, Francesco; Seferovic, Petar; Matucci-Cerinic, Marco.
Affiliation
  • Bruni C; Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Florence, Italy.
  • Buch MH; Centre for Musculoskeletal Research, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK.
  • Furst DE; NIHR Biomedical Research Centre, Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK.
  • De Luca G; Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Florence, Italy.
  • Djokovic A; Department Rheumatology, University of California, Los Angeles, Los Angeles, CA, USA.
  • Dumitru RB; Unit of Immunology, Rheumatology Allergy and Rare diseases, IRCSS San Raffaele Hospital, Vita Salute San Raffaele University, Milano, Italy.
  • Giollo A; Department of Cardiology, University Hospital Centre Bezanijska Kosa, Belgrade, Serbia.
  • Polovina M; School of Medicine, University of Belgrade, Belgrade, Serbia.
  • Steelandt A; Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK.
  • Bratis K; Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy.
  • Suliman YA; Department of Cardiology, University Hospital Centre Bezanijska Kosa, Belgrade, Serbia.
  • Milinkovic I; Rheumatology Department, Paris University, Cochin Hospital, Paris, France.
  • Baritussio A; Division of Cardiovascular Sciences, University of Manchester, Manchester, UK.
  • Hasan G; Department of Rheumatology and Rehabilitation, Faculty of Medicine, Assuit University Hospital, Assuit, Arab Republic of Egypt.
  • Xintarakou A; Department of Cardiology, University Hospital Centre Bezanijska Kosa, Belgrade, Serbia.
  • Isomura Y; Cardiology and Cardio-Immunology Outpatient Clinic, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy.
  • Markousis-Mavrogenis G; Department of Medicine, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA.
  • Tofani L; Department of Cardiology, Mitera General Hospital-Hygeia Group, Athens, Greece.
  • Mavrogeni S; Department of Allergy and Rheumatology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan.
  • Gargani L; Onassis Cardiac Surgery Centre and Kapodistrian University of Athens, Athens, Attica, Greece.
  • Caforio AL; Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Florence, Italy.
  • Tschöpe C; Onassis Cardiac Surgery Centre and Kapodistrian University of Athens, Athens, Attica, Greece.
  • Ristic A; Institute of Clinical Physiology, National Research Council, Pisa, Italy.
  • Klingel K; Cardiology and Cardio-Immunology Outpatient Clinic, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy.
  • Plein S; European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), Brussels, Belgium.
  • Behr ER; Department of Internal Medicine and Cardiology, Charité Campus Virchow-Klinikum, Charité - University Medicine Berlin, Berlin, Germany.
  • Allanore Y; Department of Cardiology, University Hospital Centre Bezanijska Kosa, Belgrade, Serbia.
  • Kuwana M; Institute for Pathology and Neuropathology, Cardiopathology, University of Tuebingen, Tuebingen, Germany.
  • Denton CP; Leeds Institute of Cardiovascular and Metabolic Medicine, University of Leeds, Leeds, UK.
  • Khanna D; Cardiology Clinical Academic Group, Institute of Molecular and Clinical Sciences, St George's University of London, London, UK.
  • Krieg T; St George's University Hospitals NHS Foundation Trust, London, UK.
  • Marcolongo R; Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy.
  • Galetti I; Department of Allergy and Rheumatology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan.
  • Zanatta E; Department Rheumatology, Royal Free Hospital, London, UK.
  • Tona F; University of Michigan Scleroderma Program, Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA.
  • Seferovic P; Translational Matrix Biology, Medical Faculty, University of Cologne, Cologne, Germany.
  • Matucci-Cerinic M; Cardiology and Cardio-Immunology Outpatient Clinic, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy.
J Scleroderma Relat Disord ; 7(1): 24-32, 2022 Feb.
Article in En | MEDLINE | ID: mdl-35386946
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.
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Full text: 1 Collection: 01-internacional Database: MEDLINE Type of study: Systematic_reviews Language: En Journal: J Scleroderma Relat Disord Year: 2022 Document type: Article Affiliation country: Italy
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Full text: 1 Collection: 01-internacional Database: MEDLINE Type of study: Systematic_reviews Language: En Journal: J Scleroderma Relat Disord Year: 2022 Document type: Article Affiliation country: Italy