Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2023 Oct;8(3):169-182.
doi: 10.1177/23971983231163413. Epub 2023 Apr 3.

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  1   2 , Maya H Buch  3   4 , Aleksandra Djokovic  5   6 , Giacomo De Luca  7 , Raluca B Dumitru  8 , Alessandro Giollo  9 , Ilaria Galetti  10 , Alexia Steelandt  11 , Konstantinos Bratis  12   13 , Yossra Atef Suliman  14 , Ivan Milinkovic  5   6 , Anna Baritussio  15 , Ghadeer Hasan  16 , Anastasia Xintarakou  17 , Yohei Isomura  18 , George Markousis-Mavrogenis  19 , Sophie Mavrogeni  20 , Luna Gargani  21 , Alida Lp Caforio  15 , Carsten Tschöpe  22 , Arsen Ristic  5   6 , Sven Plein  23 , Elijah Behr  24 , Yannick Allanore  11 , Masataka Kuwana  18 , Christopher P Denton  25 , Daniel E Furst  1   26 , Dinesh Khanna  27 , Thomas Krieg  28 , Renzo Marcolongo  15 , Alessia Pepe  29 , Oliver Distler  2 , Petros Sfikakis  30 , Petar Seferovic  5   6   31 , Marco Matucci-Cerinic  1   7
Affiliations
Review

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 et al. J Scleroderma Relat Disord. 2023 Oct.

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.

PubMed Disclaimer

Conflict of interest statement

The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Aleksandra Djokovic: no conflicts of interest to declare. Alessandro Giollo received consulting fees and/or honoraria from Novartis, Lilly, and Galapagos. Alessia Pepe: no conflicts of interest to declare. Alexia Steelandt: no conflicts of interest to declare. Alida L.P. Caforio: no conflicts of interest to declare. Anastasia Xintarakou: no conflicts of interest to declare. Anna Baritussio: no conflicts of interest to declare. Arsen Ristic: no conflicts of interest to declare. Carsten Tschöpe: no conflicts of interest to declare. Christopher P. Denton has received consultancy fees and/or research grant funding from Actelion, GlaxoSmithKline, Bayer, Sanofi-Aventis, Inventiva, Boehringer Ingelheim, Roche, CSL Behring, UCB Pharma, Leadiant Biosciences, Corbus, and Acceleron. Cosimo Bruni received consulting fees and/or honoraria from Actelion, Eli-Lilly, Boehringer Ingelheim; research grants from Gruppo Italiano Lotta alla Sclerodermia (GILS), European Scleroderma Trials and Research Group (EUSTAR), and Scleroderma Research Foundation (SRF), Scleroderma Clinical Trials Consortium (SCTC); and educational grants from AbbVie. Cosimo Bruni was supported by an Italian Ministry of University and Research PhD Scholarship, and this study is part of his PhD thesis. Daniel E. Furst reports grant/research support from Corbus, Galapagos GSK, Pfizer, Talaris, CSL Behring, Mitsubishi; Consultant fees from Actelion, Amgen, Corbus, Galapagos, Novartis, Pfizer, Roche/Genentech, Talaris, CSL Behring, and Boehringer Ingelheim. Dinesh Khanna reports grant support from NIH, Immune Tolerance Network, Bayer, BMS, Horizon, and Pfizer; consultant for Acceleron, Actelion, AbbVie, Amgen, Bayer, Boehringer Ingelheim, Chemomab, CSL Behring, Genentech/Roche, Horizon, Merck, Mitsubishi Tanabe Pharma, Prometheus Leadership/Equity position—Chief Medical Officer, and Eicos Sciences, Inc. Elijah R Behr: no conflicts of interest to declare. George Markousis-Mavrogenis: no conflicts of interest to declare. Ghadeer Hasan: no conflicts of interest to declare. Giacomo De Luca received honoraria from SOBI, Novartis, Pfizer, MSD, and Celgene. Ivan Milinkovic received honoraria/support from Boehringer Ingelheim and Hemofarm-Stada. Kostantinos Bratis: no conflicts of interest to declare. Luna Gargani received consultancy fees from GE Healthcare, Philips Healthcare, and Caption Health outside the submitted work. Marco Matucci-Cerinic received consultancies from Actelion, Janssen, Inventiva, Bayer, Biogen, Boehringer, CSL Behring, Corbus, Galapagos, Mitsubishi, Samsung, Regeneron, Acceleron, MSD, Chemomab, Lilly, Pfizer, and Roche. Masataka Kuwana received consultancy fees and/or research grant funding from AbbVie, Astellas, Bayer, Boehringer Ingelheim, Chugai, Corbus, Eisai, Horizon, Janssen, Mochida, Nippon Shinyaku, Ono Pharmaceuticals, Pfizer, and Mitsubishi Tanabe. Maya H. Buch: no conflicts of interest to declare. Oliver Distler has/had consultancy relationship with and/or has received research funding from and/or has served as a speaker for the following companies in the area of potential treatments for systemic sclerosis and its complications in the last three calendar years: 4P-Pharma, AbbVie, Acceleron, Alcimed, Amgen, AnaMar, Arxx, AstraZeneca, Baecon, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, 4P Science, Galapagos, Glenmark, Horizon, Inventiva, Janssen, Kymera, Lupin, Medscape, Miltenyi Biotec, Mitsubishi Tanabe, MSD, Novartis, Prometheus, Redxpharma, Roivant, Sanofi, and Topadur; patent issued “mir-29 for the treatment of systemic sclerosis” (US8247389, EP2331143); research grants from Kymera, Mitsubishi Tanabe. Petar Seferovic: no conflicts of interest to declare. Petros Sfikakis reports consultancy fee from Actelion, Pfizer, Genesis, MSD, UCB, Boehringer Ingelheim, Enorasis, Farmaserv, Lilly, Gliead, AbbVie, and Novartis; grants/research support from AbbVie, Roche, Pfizer, Faran, Amgen, Jannsen, Boehringer Ingelheim, and Gilead. Raluca B. Dumitru: no conflicts of interest to declare. Renzo Marcolongo: no conflicts of interest to declare. Sophie Mavrogeni: no conflicts of interest to declare. Sven Plein: no conflicts of interest to declare. Thomas Krieg reports consultancy fee and grant funding from Actelion. Yannick Allanore reports personal fees from Actelion, Bayer, BMS, Boehringer, and Curzion and grants and personal fees from Inventiva, Roche, and Sanofi. Yohei Isomura: no conflicts of interest to declare. Yossra Atef Suliman: no conflicts of interest to declare.

Figures

Figure 1.
Figure 1.
PRISMA scheme of the evaluation and selection procedure of scientific articles for the systematic review of the literature.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Varga J, Trojanowska M, Kuwana M. Pathogenesis of systemic sclerosis: recent insights of molecular and cellular mechanisms and therapeutic opportunities. J Scleroderma Relate Disorders 2017; 2(3): 137–152.
    1. De Luca G, Cavalli G, Campochiaro C, et al. Interleukin-1 and systemic sclerosis: getting to the heart of cardiac involvement. Front Immunol 2021; 12: 653950. - PMC - PubMed
    1. Caforio ALP, De Luca G, Baritussio A, et al. Serum organ-specific anti-heart and anti-intercalated disk autoantibodies as new autoimmune markers of cardiac involvement in systemic sclerosis: frequency, clinical and prognostic correlates. Diagnostics (Basel) 2021; 11(11): 2165. - PMC - PubMed
    1. Elhai M, Meune C, Boubaya M, et al. Mapping and predicting mortality from systemic sclerosis. Ann Rheum Dis 2017; 76(11): 1897–1905. - PubMed
    1. Hao Y, Hudson M, Baron M, et al. Early mortality in a multinational systemic sclerosis inception cohort. Arthritis Rheumatol 2017; 69(5): 1067–1077. - PubMed

LinkOut - more resources