{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T11:52:03Z","timestamp":1788609123774,"version":"build-2803163510"},"reference-count":143,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:sec>\n            <jats:title>Purpose of review<\/jats:title>\n            <jats:p>In systemic sclerosis (SSc) primary heart involvement (pHI) is frequent, even though often unrecognized due to its occult nature and to the lack of a specific diagnostic algorithm. The purpose of this review is to report the state of the art of the evidence in the current literature, as well as the overall diagnostic modalities and therapeutic strategies for primary heart involvement in SSc.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Recent findings<\/jats:title>\n            <jats:p>SSc-pHI is defined by the presence of cardiac abnormalities that are predominantly attributable to SSc rather than other causes and\/or complications; it may be sub-clinical and must be confirmed through diagnostic investigations. Novel electrocardiographic analysis and cardiac magnetic resonance (CMR) with mapping techniques have been recently proposed, showing a great utility in the early identification of SSc-pHI and in the noninvasive characterization of myocardial tissue. Immunosuppressive therapy emerged as fundamental to curb myocardial inflammation, and recent preclinical and clinical data support the role of antifibrotic drugs to treat SSc-pHI.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Summary<\/jats:title>\n            <jats:p>our review will help clinicians to properly integrate the available diagnostic modalities for the assessment of SSc-pHI. The ultimate goal is to propose a feasible diagnostic algorithm for the early identification of patients with SSc-pHI, and a schematic therapeutic approach to manage SSc-pHI.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1097\/bor.0000000000000990","type":"journal-article","created":{"date-parts":[[2023,11,14]],"date-time":"2023-11-14T13:10:56Z","timestamp":1699967456000},"page":"76-93","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":22,"title":["Diagnosis and management of primary heart involvement in systemic sclerosis"],"prefix":"10.1097","volume":"36","author":[{"given":"Giacomo","family":"De Luca","sequence":"first","affiliation":[{"name":"Vita-Salute San Raffaele University"},{"name":"Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Matucci-Cerinic","sequence":"additional","affiliation":[{"name":"Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan"},{"name":"Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie I.","family":"Mavrogeni","sequence":"additional","affiliation":[{"name":"Onassis Cardiac Surgery Center"},{"name":"University Research Institute of Maternal and Child Health and Precision Medicine and UNESCO Chair in Adolescent Healthcare, Medical School, National and Kapodistrian University of Athens, Aghia Sophia Children's Hospital, Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2023,11,8]]},"reference":[{"key":"R1-20250526","doi-asserted-by":"crossref","first-page":"1685","DOI":"10.1016\/S0140-6736(17)30933-9","article-title":"Systemic sclerosis","volume":"390","author":"Denton","year":"2017","journal-title":"Lancet"},{"key":"R2-20250526","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/j.rdc.2007.12.002","article-title":"The heart in scleroderma","volume":"34","author":"Champion","year":"2008","journal-title":"Rheum Dis Clin North Am"},{"key":"R3-20250526","doi-asserted-by":"crossref","first-page":"101668","DOI":"10.1016\/j.berh.2021.101668","article-title":"Cardiac involvement in systemic sclerosis: getting to the heart of the matter","volume":"35","author":"Bruni","year":"2021","journal-title":"Best Pract Res Clin Rheumatol"},{"key":"R4-20250526","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 Systemic sclerosis myocarditis EULAR Scleroderma Trials and Research (EUSTAR) database","volume":"69","author":"Tyndall","year":"2010","journal-title":"Ann Rheum Dis"},{"key":"R5-20250526","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1016\/j.acvd.2009.06.009","article-title":"Heart involvement in systemic sclerosis: evolving concept and diagnostic methodologies","volume":"103","author":"Meune","year":"2010","journal-title":"Arch Cardiovasc Dis"},{"key":"R6-20250526","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"},{"key":"R7-20250526","first-page":"428e40","article-title":"Pathologic observations in systemic sclerosis (scleroderma): a study of fifty-eight autopsy cases and fifty-eight matched controls","volume":"46","author":"D\u2019Angelo","year":"1969","journal-title":"Am J Med"},{"key":"R8-20250526","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1161\/01.CIR.53.3.483","article-title":"Myocardial lesions of progressive systemic sclerosis. 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