{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,2]],"date-time":"2026-11-02T08:29:36Z","timestamp":1793608176501,"version":"4.5.0"},"reference-count":52,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2026,3,23]],"date-time":"2026-03-23T00:00:00Z","timestamp":1774224000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000957","name":"Alzheimer's Association","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000957","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Best Practice &amp; Research Clinical Rheumatology"],"published-print":{"date-parts":[[2026,9]]},"DOI":"10.1016\/j.berh.2026.102134","type":"journal-article","created":{"date-parts":[[2026,3,20]],"date-time":"2026-03-20T15:43:47Z","timestamp":1774021427000},"page":"102134","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":0,"title":["Definition, diagnosis, treatment for scleroderma primary heart involvement"],"prefix":"10.1016","volume":"40","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9224-1391","authenticated-orcid":false,"given":"DeAnna","family":"Baker Frost","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryan J.","family":"Tedford","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Silver","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"6","key":"10.1016\/j.berh.2026.102134_bib1","doi-asserted-by":"crossref","first-page":"912","DOI":"10.1093\/rheumatology\/kew488","article-title":"Consensus best practice pathway of the UK systemic sclerosis study group: management of cardiac disease in systemic sclerosis","volume":"56","author":"Bissell","year":"2017","journal-title":"Rheumatology (Oxford, England)"},{"key":"10.1016\/j.berh.2026.102134_bib2","unstructured":"Tonutti A, Motta F, De Angelis R, Cipolletta E, Ferri C, Bajocchi G, et al. First-line diagnostic tests to intercept primary heart involvement in systemic sclerosis: clinical associations from the SPRING-SIR registry. Eur J Clin Invest.n\/a(n\/a):e70094."},{"issue":"6","key":"10.1016\/j.berh.2026.102134_bib3","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1001\/archinte.1943.00210060010002","article-title":"Scleroderma heart disease: with a consideration of certain other visceral manifestations of scleroderm","volume":"71","author":"Weiss","year":"1943","journal-title":"Arch Intern Med"},{"issue":"3","key":"10.1016\/j.berh.2026.102134_bib4","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1161\/01.CIR.53.3.483","article-title":"Myocardial lesions of progressive systemic sclerosis. A cause of cardiac dysfunction","volume":"53","author":"Bulkley","year":"1976","journal-title":"Circulation"},{"issue":"5","key":"10.1016\/j.berh.2026.102134_bib5","doi-asserted-by":"crossref","first-page":"913","DOI":"10.1016\/0002-8703(81)90043-0","article-title":"Primary heart disease in systemic sclerosis (Scleroderma): advances in clinical and pathologic features, pathogenesis, and new therapeutic approaches","volume":"102","author":"Botstein","year":"1981","journal-title":"Am Heart J"},{"issue":"8","key":"10.1016\/j.berh.2026.102134_bib6","first-page":"1516","article-title":"The burden of illness of the organ manifestations of systemic sclerosis: a pragmatic, targeted review","volume":"43","author":"Smith","year":"2025","journal-title":"Clin Exp Rheumatol"},{"issue":"11","key":"10.1016\/j.berh.2026.102134_bib7","doi-asserted-by":"crossref","first-page":"1897","DOI":"10.1136\/annrheumdis-2017-211448","article-title":"Mapping and predicting mortality from systemic sclerosis","volume":"76","author":"Elhai","year":"2017","journal-title":"Ann Rheum Dis"},{"issue":"10","key":"10.1016\/j.berh.2026.102134_bib8","doi-asserted-by":"crossref","first-page":"1809","DOI":"10.1136\/ard.2009.114264","article-title":"Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma trials and research (EUSTAR) database","volume":"69","author":"Tyndall","year":"2010","journal-title":"Ann Rheum Dis"},{"issue":"12","key":"10.1016\/j.berh.2026.102134_bib9","doi-asserted-by":"crossref","first-page":"1878","DOI":"10.1136\/ard.2008.095836","article-title":"Cardiac magnetic resonance imaging in systemic sclerosis: a cross-sectional observational study of 52 patients","volume":"68","author":"Hachulla","year":"2009","journal-title":"Ann Rheum Dis"},{"issue":"2","key":"10.1016\/j.berh.2026.102134_bib10","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1515\/rir-2024-0010","article-title":"Therapeutic strategies for primary heart involvement in systemic sclerosis","volume":"5","author":"Batani","year":"2024","journal-title":"Rheumatol Immunol Res"},{"issue":"3","key":"10.1016\/j.berh.2026.102134_bib11","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1080\/1744666X.2023.2171988","article-title":"Understanding and managing cardiac involvement in systemic sclerosis","volume":"19","author":"Moysidou","year":"2023","journal-title":"Expet Rev Clin Immunol"},{"issue":"8","key":"10.1016\/j.berh.2026.102134_bib12","doi-asserted-by":"crossref","first-page":"3646","DOI":"10.1093\/rheumatology\/keaa827","article-title":"Health-related quality of life in patients with systemic sclerosis: evolution over time and main determinants","volume":"60","author":"van Leeuwen","year":"2021","journal-title":"Rheumatology"},{"issue":"1","key":"10.1016\/j.berh.2026.102134_bib13","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1177\/23971983211053246","article-title":"Primary systemic sclerosis heart involvement: a systematic literature review and preliminary data-driven, consensus-based WSF\/HFA definition","volume":"7","author":"Bruni","year":"2022","journal-title":"J scleroderma relat disord"},{"issue":"6","key":"10.1016\/j.berh.2026.102134_bib14","doi-asserted-by":"crossref","first-page":"2934","DOI":"10.1093\/rheumatology\/keaa742","article-title":"Predictors of subclinical systemic sclerosis primary heart involvement characterised by microvasculopathy and myocardial fibrosis","volume":"60","author":"Dumitru","year":"2020","journal-title":"Rheumatology"},{"key":"10.1016\/j.berh.2026.102134_bib15","doi-asserted-by":"crossref","DOI":"10.1016\/j.semarthrit.2024.152622","article-title":"Immunosuppressive therapy to treat newly diagnosed primary heart involvement in patients with systemic sclerosis: an Italian cardiac magnetic resonance based study","volume":"71","author":"De Luca","year":"2025","journal-title":"Semin Arthritis Rheum"},{"issue":"3","key":"10.1016\/j.berh.2026.102134_bib16","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1177\/23971983231163413","article-title":"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","volume":"8","author":"Bruni","year":"2023","journal-title":"J scleroderma relat disord"},{"issue":"11","key":"10.1016\/j.berh.2026.102134_bib17","doi-asserted-by":"crossref","first-page":"1576","DOI":"10.1136\/annrheumdis-2019-215486","article-title":"Vasodilators and low-dose acetylsalicylic acid are associated with a lower incidence of distinct primary myocardial disease manifestations in systemic sclerosis: results of the DeSScipher inception cohort study","volume":"78","author":"Valentini","year":"2019","journal-title":"Ann Rheum Dis"},{"issue":"26","key":"10.1016\/j.berh.2026.102134_bib18","doi-asserted-by":"crossref","first-page":"2590","DOI":"10.1093\/eurheartj\/ehaa857","article-title":"Assessment and pathophysiology of microvascular disease: recent progress and clinical implications","volume":"42","author":"Masi","year":"2021","journal-title":"Eur Heart J"},{"key":"10.1016\/j.berh.2026.102134_bib19","doi-asserted-by":"crossref","DOI":"10.1177\/23971983241313096","article-title":"Cardiac involvement in systemic sclerosis: a critical review of knowledge gaps and opportunities","author":"Jones","year":"2025","journal-title":"J scleroderma relat disord"},{"key":"10.1016\/j.berh.2026.102134_bib20","doi-asserted-by":"crossref","DOI":"10.3389\/fmed.2025.1606593","article-title":"Cardiac magnetic resonance in systemic sclerosis: imaging features and potential prognostic implications. A literature review","volume":"12","author":"Vitale","year":"2025","journal-title":"Front Med"},{"issue":"1","key":"10.1016\/j.berh.2026.102134_bib21","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1016\/j.rdc.2013.10.007","article-title":"Systemic sclerosis and the heart","volume":"40","author":"Parks","year":"2014","journal-title":"Rheum Dis Clin N Am"},{"issue":"18","key":"10.1016\/j.berh.2026.102134_bib22","doi-asserted-by":"crossref","DOI":"10.3390\/jcm14186383","article-title":"Predictive value of classical and emerging autoantibodies for cardiac dysfunction in systemic sclerosis: systematic review","volume":"14","author":"Radi\u0107","year":"2025","journal-title":"J Clin Med"},{"issue":"7","key":"10.1016\/j.berh.2026.102134_bib23","doi-asserted-by":"crossref","first-page":"1172","DOI":"10.1093\/rheumatology\/ket377","article-title":"Cardiac arrhythmias and conduction defects in systemic sclerosis","volume":"53","author":"Vacca","year":"2014","journal-title":"Rheumatology (Oxford, England)"},{"issue":"10","key":"10.1016\/j.berh.2026.102134_bib24","article-title":"Arrhythmias in systemic sclerosis: a call for interdisciplinarity teams","volume":"15","author":"Costan","year":"2025","journal-title":"Life (Basel)"},{"issue":"17","key":"10.1016\/j.berh.2026.102134_bib25","doi-asserted-by":"crossref","DOI":"10.1161\/JAHA.119.013405","article-title":"Cardiovascular manifestations of systemic sclerosis: a Danish nationwide cohort study","volume":"8","author":"Butt","year":"2019","journal-title":"J Am Heart Assoc"},{"issue":"9","key":"10.1016\/j.berh.2026.102134_bib26","doi-asserted-by":"crossref","first-page":"2523","DOI":"10.1093\/rheumatology\/kez658","article-title":"Systemic sclerosis myocarditis has unique clinical, histological and prognostic features: a comparative histological analysis","volume":"59","author":"De Luca","year":"2020","journal-title":"Rheumatology (Oxford, England)"},{"issue":"5","key":"10.1016\/j.berh.2026.102134_bib27","doi-asserted-by":"crossref","DOI":"10.1097\/RHU.0000000000001039","article-title":"Myocarditis in scleroderma: an underrecognized manifestation of the disease","volume":"26","author":"Theodorou","year":"2020","journal-title":"J Clin Rheumatol : practical reports on rheumatic & musculoskeletal diseases"},{"issue":"10","key":"10.1016\/j.berh.2026.102134_bib28","doi-asserted-by":"crossref","first-page":"1823","DOI":"10.1007\/s00296-024-05699-x","article-title":"Heart involvement in patients with systemic sclerosis-what have we learned about it in the last 5 years","volume":"44","author":"Nadel","year":"2024","journal-title":"Rheumatol Int"},{"issue":"4","key":"10.1016\/j.berh.2026.102134_bib29","doi-asserted-by":"crossref","DOI":"10.1002\/pul2.70215","article-title":"Progressive pericardial effusions (PEF) in patients with pulmonary arterial hypertension (PAH) taking sotatercept-association with pre-existing pericardial effusion","volume":"15","author":"Subba","year":"2025","journal-title":"Pulm Circ"},{"issue":"5","key":"10.1016\/j.berh.2026.102134_bib30","doi-asserted-by":"crossref","first-page":"849","DOI":"10.1002\/art.42054","article-title":"Diastolic dysfunction in systemic sclerosis: risk factors and impact on mortality","volume":"74","author":"Hinze","year":"2022","journal-title":"Arthritis & rheumatology (Hoboken, NJ)"},{"issue":"5","key":"10.1016\/j.berh.2026.102134_bib31","doi-asserted-by":"crossref","DOI":"10.1161\/JAHA.124.036730","article-title":"Evaluation of systemic sclerosis primary heart involvement and chronic heart failure in the european Scleroderma trials and research cohort","volume":"14","author":"Gy\u00f6rfi","year":"2025","journal-title":"J Am Heart Assoc"},{"issue":"1","key":"10.1016\/j.berh.2026.102134_bib32","doi-asserted-by":"crossref","first-page":"17","DOI":"10.2217\/fca-2020-0238","article-title":"Prevalence and prognostic significance of heart failure with preserved ejection fraction in systemic sclerosis","volume":"18","author":"Fontes Oliveira","year":"2022","journal-title":"Future Cardiol"},{"issue":"7","key":"10.1016\/j.berh.2026.102134_bib33","doi-asserted-by":"crossref","first-page":"1047","DOI":"10.3899\/jrheum.201005","article-title":"Increased risk of valvular heart disease in systemic sclerosis: an underrecognized cardiac complication","volume":"48","author":"Kurmann","year":"2021","journal-title":"J Rheumatol"},{"issue":"3","key":"10.1016\/j.berh.2026.102134_bib34","doi-asserted-by":"crossref","first-page":"394","DOI":"10.3899\/jrheum.201025","article-title":"Increased prevalence of moderate to severe mitral and aortic valve dysfunction in systemic sclerosis: a case-control study","volume":"48","author":"Narv\u00e1ez","year":"2021","journal-title":"J Rheumatol"},{"issue":"24","key":"10.1016\/j.berh.2026.102134_bib35","doi-asserted-by":"crossref","first-page":"3177","DOI":"10.2169\/internalmedicine.5226-20","article-title":"Premature onset aortic stenosis in systemic sclerosis: a report of a series of cases","volume":"59","author":"Machida","year":"2020","journal-title":"Internal medicine (Tokyo, Japan)"},{"issue":"10","key":"10.1016\/j.berh.2026.102134_bib36","first-page":"1933","article-title":"Troponin elevation independently associates with mortality in systemic sclerosis","volume":"40","author":"Paik","year":"2022","journal-title":"Clin Exp Rheumatol"},{"issue":"9","key":"10.1016\/j.berh.2026.102134_bib37","doi-asserted-by":"crossref","first-page":"5004","DOI":"10.1093\/rheumatology\/keaf234","article-title":"The complex diagnosis of primary heart involvement in systemic sclerosis: red flags to drive the choice of cardiac magnetic resonance","volume":"64","author":"Batani","year":"2025","journal-title":"Rheumatology (Oxford, England)"},{"key":"10.1016\/j.berh.2026.102134_bib38","article-title":"Individualized care or standardized protocol: should all patients with systemic sclerosis receive cardiac MRI?","author":"Csecs","year":"2025","journal-title":"Rheumatology"},{"issue":"4","key":"10.1016\/j.berh.2026.102134_bib39","doi-asserted-by":"crossref","first-page":"1535","DOI":"10.1093\/rheumatology\/keac504","article-title":"Cardiac magnetic resonance imaging before and after therapeutic interventions for systemic sclerosis-associated myocarditis","volume":"62","author":"Panopoulos","year":"2023","journal-title":"Rheumatology (Oxford, England)"},{"key":"10.1016\/j.berh.2026.102134_bib40","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1016\/j.amjcard.2020.03.041","article-title":"Usefulness of Interleukin-1 receptor antagonists in patients with recurrent pericarditis","volume":"127","author":"Khayata","year":"2020","journal-title":"Am J Cardiol"},{"issue":"113","key":"10.1016\/j.berh.2026.102134_bib41","first-page":"146","article-title":"Randomised, double-blind, placebo-controlled trial of IL1-trap, rilonacept, in systemic sclerosis. A phase I\/II biomarker trial","volume":"36","author":"Mantero","year":"2018","journal-title":"Clin Exp Rheumatol"},{"issue":"9","key":"10.1016\/j.berh.2026.102134_bib42","doi-asserted-by":"crossref","first-page":"708","DOI":"10.1016\/S2213-2600(16)30152-7","article-title":"Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial","volume":"4","author":"Tashkin","year":"2016","journal-title":"Lancet Respir Med"},{"issue":"7","key":"10.1016\/j.berh.2026.102134_bib43","doi-asserted-by":"crossref","DOI":"10.1136\/annrheumdis-2019-216070","article-title":"Anti-Ku syndrome with elevated CK: association with myocardial involvement in systemic sclerosis","volume":"80","author":"Campochiaro","year":"2021","journal-title":"Ann Rheum Dis"},{"issue":"7","key":"10.1016\/j.berh.2026.102134_bib44","doi-asserted-by":"crossref","first-page":"594","DOI":"10.1016\/j.revmed.2017.12.013","article-title":"[Systemic sclerosis: efficacy of intravenous immunoglobulins in severe cardiac involvement?]","volume":"39","author":"Cacciatore","year":"2018","journal-title":"Rev Med Interne"},{"issue":"3","key":"10.1016\/j.berh.2026.102134_bib45","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1097\/BOR.0000000000000795","article-title":"Treatment for systemic sclerosis-associated interstitial lung disease","volume":"33","author":"Roofeh","year":"2021","journal-title":"Curr Opin Rheumatol"},{"issue":"8s","key":"10.1016\/j.berh.2026.102134_bib46","doi-asserted-by":"crossref","first-page":"S476","DOI":"10.1097\/RHU.0000000000001194","article-title":"Tocilizumab for the treatment of myocardial inflammation shown by cardiac magnetic resonance: report of two cases and rationale for its therapeutic use","volume":"27","author":"Campochiaro","year":"2021","journal-title":"J Clin Rheumatol : practical reports on rheumatic & musculoskeletal diseases"},{"issue":"6","key":"10.1016\/j.berh.2026.102134_bib47","doi-asserted-by":"crossref","first-page":"e205","DOI":"10.1093\/rheumatology\/keaa865","article-title":"Treatment of myocardial fibrosis in systemic sclerosis with tocilizumab","volume":"60","author":"Ishizaki","year":"2021","journal-title":"Rheumatology"},{"issue":"10","key":"10.1016\/j.berh.2026.102134_bib48","first-page":"2006","article-title":"Tocilizumab for systemic sclerosis with cardiac involvement: a case report","volume":"40","author":"Lee","year":"2022","journal-title":"Clin Exp Rheumatol"},{"issue":"26","key":"10.1016\/j.berh.2026.102134_bib49","doi-asserted-by":"crossref","first-page":"2518","DOI":"10.1056\/NEJMoa1903076","article-title":"Nintedanib for systemic sclerosis-associated interstitial lung disease","volume":"380","author":"Distler","year":"2019","journal-title":"N Engl J Med"},{"issue":"7","key":"10.1016\/j.berh.2026.102134_bib50","doi-asserted-by":"crossref","first-page":"2550","DOI":"10.1093\/rheumatology\/keac674","article-title":"Beneficial effects of nintedanib on cardiomyopathy in patients with systemic sclerosis: a pilot study","volume":"62","author":"Ninagawa","year":"2023","journal-title":"Rheumatology (Oxford, England)"},{"issue":"8","key":"10.1016\/j.berh.2026.102134_bib51","doi-asserted-by":"crossref","first-page":"1117","DOI":"10.1136\/ard-2023-223952","article-title":"Treatment of a patient with severe systemic sclerosis (SSc) using CD19-targeted CAR T cells","volume":"82","author":"Bergmann","year":"2023","journal-title":"Ann Rheum Dis"},{"issue":"18","key":"10.1016\/j.berh.2026.102134_bib52","first-page":"e895","article-title":"AHA\/ACC\/HFSA guideline for the management of heart failure: a report of the American college of cardiology\/American heart association joint committee on clinical practice guidelines","volume":"145","author":"Heidenreich","year":"2022","journal-title":"Circulation"}],"container-title":["Best Practice &amp; Research Clinical Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1521694226000197?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1521694226000197?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,11,2]],"date-time":"2026-11-02T08:11:45Z","timestamp":1793607105000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1521694226000197"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9]]},"references-count":52,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2026,9]]}},"alternative-id":["S1521694226000197"],"URL":"https:\/\/doi.org\/10.1016\/j.berh.2026.102134","relation":{},"ISSN":["1521-6942"],"issn-type":[{"value":"1521-6942","type":"print"}],"subject":[],"published":{"date-parts":[[2026,9]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Definition, diagnosis, treatment for scleroderma primary heart involvement","name":"articletitle","label":"Article Title"},{"value":"Best Practice & Research Clinical Rheumatology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.berh.2026.102134","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2026 The Authors. Published by Elsevier Ltd.","name":"copyright","label":"Copyright"}],"article-number":"102134"}}