Consensus on the assessment of systemic sclerosis-associated primary heart involvement: World Scleroderma Foundation/Heart Failure Association guidance on screening, diagnosis, and follow-up assessment

Cosimo Bruni; Maya H Buch; Aleksandra Djokovic; Giacomo De Luca; Raluca B Dumitru; Alessandro Giollo; Ilaria Galetti; Alexia Steelandt; Konstantinos Bratis; Yossra Atef Suliman; Ivan Milinkovic; Anna Baritussio; Ghadeer Hasan; Anastasia Xintarakou; Yohei Isomura; George Markousis-Mavrogenis; Sophie Mavrogeni; Luna Gargani; Alida Lp Caforio; Carsten Tschöpe; Arsen Ristic; Sven Plein; Elijah Behr; Yannick Allanore; Masataka Kuwana; Christopher P Denton; Daniel E Furst; Dinesh Khanna; Thomas Krieg; Renzo Marcolongo; Alessia Pepe; Oliver Distler; Petros Sfikakis; Petar Seferovic; Marco Matucci-Cerinic

doi:10.1177/23971983231163413

Consensus on the assessment of systemic sclerosis-associated primary heart involvement: World Scleroderma Foundation/Heart Failure Association guidance on screening, diagnosis, and follow-up assessment

J Scleroderma Relat Disord. 2023 Oct;8(3):169-182. doi: 10.1177/23971983231163413. Epub 2023 Apr 3.

Authors

Cosimo Bruni^{1

2}, Maya H Buch^{3

4}, Aleksandra Djokovic^{5

6}, Giacomo De Luca⁷, Raluca B Dumitru⁸, Alessandro Giollo⁹, Ilaria Galetti¹⁰, Alexia Steelandt¹¹, Konstantinos Bratis^{12

13}, Yossra Atef Suliman¹⁴, Ivan Milinkovic^{5

6}, Anna Baritussio¹⁵, Ghadeer Hasan¹⁶, Anastasia Xintarakou¹⁷, Yohei Isomura¹⁸, George Markousis-Mavrogenis¹⁹, Sophie Mavrogeni²⁰, Luna Gargani²¹, Alida Lp Caforio¹⁵, Carsten Tschöpe²², Arsen Ristic^{5

6}, Sven Plein²³, Elijah Behr²⁴, Yannick Allanore¹¹, Masataka Kuwana¹⁸, Christopher P Denton²⁵, Daniel E Furst^{1

26}, Dinesh Khanna²⁷, Thomas Krieg²⁸, Renzo Marcolongo¹⁵, Alessia Pepe²⁹, Oliver Distler², Petros Sfikakis³⁰, Petar Seferovic^{5

6

31}, Marco Matucci-Cerinic^{1

7}

Affiliations

¹ Division of Rheumatology, Department of Experimental and Clinical Medicine, Careggi University Hospital (AOUC), University of Florence, Florence, Italy.
² Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
³ Centre for Musculoskeletal Research, School of Biological Sciences, Faculty of Biology, Medicine & Health, University of Manchester, Manchester, UK.
⁴ NIHR Manchester Biomedical Research Centre, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK.
⁵ Department of Cardiology, University Clinical Hospital Center Bezanijska kosa, Belgrade, Serbia.
⁶ Faculty of Medicine, University of Belgrade, Belgrade, Serbia.
⁷ Unit of Immunology, Rheumatology Allergology and Rare diseases, IRCSS San Raffaele Hospital, Milan, Italy.
⁸ Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK.
⁹ Rheumatology Unit, Department of Medicine, University of Padova, Padova, Italy.
¹⁰ Federation of European Scleroderma Associations (FESCA), Saint Maur, Belgium.
¹¹ Rheumatology Department, Paris University, Cochin Hospital, Paris, France.
¹² Manchester Heart Centre, Manchester University NHS Foundation Trust, Manchester, UK.
¹³ Division of Cardiovascular Sciences, University of Manchester, Manchester, UK.
¹⁴ Department of Rheumatology and Rehabilitation, Faculty of Medicine, Assuit University Hospital, Assuit, Arab Republic of Egypt.
¹⁵ Cardiology and Cardioimmunology Outpatient Clinic, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy.
¹⁶ Department of Medicine, Rutgers Robert Wood Johnson Medical Centre, New Brunswick, NJ, USA.
¹⁷ Mitera General Hospital-Hygeia Group, Athens, Greece.
¹⁸ Department of Allergy and Rheumatology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan.
¹⁹ Department of Cardiology, Onassis Cardiac Surgery Centre, Athens, Greece.
²⁰ Onassis Cardiac Surgery Centre and Kapodistrian University of Athens, Athens, Greece.
²¹ Department of Surgical, Medical and Molecular Pathology and Critical Care Medicine, University of Pisa, Pisa, Italy.
²² Department of Internal Medicine and Cardiology, Charité-University Medicine Berlin, Berlin, Germany.
²³ Biomedical Imaging Science Department, Leeds Institute of Cardiovascular and Metabolic Medicine, University of Leeds, Leeds, UK.
²⁴ Cardiovascular Clinical Academic Group, Institute of Molecular and Clinical Sciences, St George's University of London, London, UK.
²⁵ Centre for Rheumatology, Royal Free Hospital, London, UK.
²⁶ University of California, Los Angeles, Los Angeles, CA, USA.
²⁷ Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI, USA.
²⁸ Department of Dermatology, Translational Matrix Biology, University Hospital Cologne, Cologne, Germany.
²⁹ Institute of Radiology and Department of Medicine, University of Padova, Padova, Italy.
³⁰ First Department of Propaedeutic and Internal Medicine and Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece.
³¹ Serbian Academy of Sciences and Arts, Belgrade, Serbia.

Abstract

Introduction: Heart involvement is a common problem in systemic sclerosis. Recently, a definition of systemic sclerosis primary heart involvement had been proposed. Our aim was to establish consensus guidance on the screening, diagnosis and follow-up of systemic sclerosis primary heart involvement patients.

Methods: A systematic literature review was performed to investigate the tests used to evaluate heart involvement in systemic sclerosis. The extracted data were categorized into relevant domains (conventional radiology, electrocardiography, echocardiography, cardiac magnetic resonance imaging, laboratory, and others) and presented to experts and one patient research partner, who discussed the data and added their opinion. This led to the formulation of overarching principles and guidance statements, then reviewed and voted on for agreement. Consensus was attained when the mean agreement was ⩾7/10 and of ⩾70% of voters.

Results: Among 2650 publications, 168 met eligibility criteria; the data extracted were discussed over three meetings. Seven overarching principles and 10 guidance points were created, revised and voted on. The consensus highlighted the importance of patient counseling, differential diagnosis and multidisciplinary team management, as well as defining screening and diagnostic approaches. The initial core evaluation should integrate history, physical examination, rest electrocardiography, trans-thoracic echocardiography and standard serum cardiac biomarkers. Further investigations should be individually tailored and decided through a multidisciplinary management. The overall mean agreement was 9.1/10, with mean 93% of experts voting above 7/10.

Conclusion: This consensus-based guidance on screening, diagnosis and follow-up of systemic sclerosis primary heart involvement provides a foundation for standard of care and future feasibility studies that are ongoing to support its application in clinical practice.

Keywords: Systemic sclerosis; cardiac involvement; consensus guidance; diagnosis; follow-up; screening.

Publication types

Review