{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T13:18:58Z","timestamp":1774444738003,"version":"3.50.1"},"reference-count":18,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2026,3,24]],"date-time":"2026-03-24T00:00:00Z","timestamp":1774310400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Life"],"abstract":"<jats:p>Cardiologists consider degenerative or infectious causes when evaluating valvular heart disease. However, the role of autoimmune disorders, though less frequent, remains clinically significant. This report describes a young male patient presenting with persistent coronary disease and a suspected valvular cusp perforation initially attributed to infective endocarditis, which ultimately proved to be a manifestation of IgG4-related disease. IgG4-related disease is a rare condition, more prevalent in Asian populations, that typically affects the pancreas, salivary glands, lacrimal glands, and the retroperitoneum. Cardiac involvement, although uncommon, can present in various ways, including pericarditis, pulmonary arterial hypertension, valve dysfunction, cardiac pseudotumor, and coronary disease. Diagnosing and managing IgG4-related cardiac involvement requires heightened clinical suspicion, serological and histopathological assessment, and prompt interdisciplinary collaboration. Notably, involving rheumatologists in the management of these rare yet impactful autoimmune cardiac diseases is essential.<\/jats:p>","DOI":"10.3390\/life16040537","type":"journal-article","created":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T10:07:13Z","timestamp":1774433233000},"page":"537","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Beyond the Usual Suspects: IgG4-Related Disease as a Rare Culprit in Cardiac Valvular Disorders"],"prefix":"10.3390","volume":"16","author":[{"given":"Piera","family":"Costanzo","sequence":"first","affiliation":[{"name":"Department of Clinical and Biological Sciences, University of Turin, 10125 Turin, Italy"},{"name":"SC of Cardiology, San Giovanni Bosco Hospital, ASL Citt\u00e0 di Torino, 10154 Turin, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1266-9441","authenticated-orcid":false,"given":"Savino","family":"Sciascia","sequence":"additional","affiliation":[{"name":"Department of Clinical and Biological Sciences, University of Turin, 10125 Turin, Italy"},{"name":"University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, ASL Citt\u00e0 di Torino, San Giovanni Bosco Hub Hospital, 10154 Turin, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giacomo","family":"Quattrocchio","sequence":"additional","affiliation":[{"name":"University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, ASL Citt\u00e0 di Torino, San Giovanni Bosco Hub Hospital, 10154 Turin, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pierluigi","family":"Sbarra","sequence":"additional","affiliation":[{"name":"SC of Cardiology, San Giovanni Bosco Hospital, ASL Citt\u00e0 di Torino, 10154 Turin, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3566-1995","authenticated-orcid":false,"given":"Antonella","family":"Barreca","sequence":"additional","affiliation":[{"name":"Division of Pathology, Azienda Ospedaliero-Universitaria Citt\u00e0 della Salute e della Scienza,  Department of Medical Sciences, University of Turin, 10126 Turin, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roberta","family":"Bracci","sequence":"additional","affiliation":[{"name":"Department of Clinical and Biological Sciences, University of Turin, 10125 Turin, Italy"},{"name":"University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, ASL Citt\u00e0 di Torino, San Giovanni Bosco Hub Hospital, 10154 Turin, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1858-5452","authenticated-orcid":false,"given":"Irene","family":"Cecchi","sequence":"additional","affiliation":[{"name":"Department of Clinical and Biological Sciences, University of Turin, 10125 Turin, Italy"},{"name":"University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, ASL Citt\u00e0 di Torino, San Giovanni Bosco Hub Hospital, 10154 Turin, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1941-2606","authenticated-orcid":false,"given":"Massimo","family":"Radin","sequence":"additional","affiliation":[{"name":"Department of Clinical and Biological Sciences, University of Turin, 10125 Turin, Italy"},{"name":"University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, ASL Citt\u00e0 di Torino, San Giovanni Bosco Hub Hospital, 10154 Turin, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7849-6323","authenticated-orcid":false,"given":"Elisa","family":"Menegatti","sequence":"additional","affiliation":[{"name":"Department of Clinical and Biological Sciences, University of Turin, 10125 Turin, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4475-4117","authenticated-orcid":false,"given":"Simone","family":"Baldovino","sequence":"additional","affiliation":[{"name":"Department of Clinical and Biological Sciences, University of Turin, 10125 Turin, Italy"},{"name":"SC Transplant Coordinating Service, Azienda Ospedaliero-Universitaria Citt\u00e0 della Salute e della Scienza, 10126 Turin, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2026,3,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"614","DOI":"10.1038\/s41584-024-01149-x","article-title":"Current understanding and management of cardiovascular involvement in rheumatic immune-mediated inflammatory diseases","volume":"20","author":"Buch","year":"2024","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"iii123","DOI":"10.1093\/rheumatology\/kez667","article-title":"IgG4-related disease: A clinical perspective","volume":"59","author":"Maritati","year":"2020","journal-title":"Rheumatology"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"100074","DOI":"10.1016\/j.jtauto.2020.100074","article-title":"IgG4-related disease: Changing epidemiology and new thoughts on a multisystem disease","volume":"4","author":"Floreani","year":"2020","journal-title":"J. Transl. Autoimmun."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"ytac153","DOI":"10.1093\/ehjcr\/ytac153","article-title":"Cardiac manifestations of IgG4-related disease: A case series","volume":"6","author":"Ratwatte","year":"2022","journal-title":"Eur. Heart J. Case Rep."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1181","DOI":"10.1038\/modpathol.2012.72","article-title":"Consensus statement on the pathology of IgG4-related disease","volume":"25","author":"Deshpande","year":"2012","journal-title":"Mod. Pathol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"702","DOI":"10.1038\/s41584-020-0500-7","article-title":"IgG4-related disease: An update on pathophysiology and implications for clinical care","volume":"16","author":"Perugino","year":"2020","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"790","DOI":"10.1136\/heartjnl-2020-318041","article-title":"The enigmatic immunoglobulin G4-related disease and its varied cardiovascular manifestations","volume":"107","author":"Shakir","year":"2021","journal-title":"Heart"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1186\/s13019-025-03675-5","article-title":"Aortic regurgitation and combined ventricular tachycardia due to IgG4-related disease","volume":"20","author":"Hyun","year":"2025","journal-title":"J. Cardiothorac. Surg."},{"key":"ref_9","doi-asserted-by":"crossref","unstructured":"Yan, S., Peng, Y., Xu, J., Liu, Y., Li, Z., Yang, Y., Peng, L., Zhou, J., Li, L., and Liu, C. (2025). Genome-wide association studies reveal new insights into the genetic basis of IgG4-related disease in the Chinese Han population. Ann. Rheum. Dis., 1\u201311.","DOI":"10.1016\/j.ard.2025.10.028"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1151","DOI":"10.1136\/annrheumdis-2018-214819","article-title":"2019 European League Against Rheumatism\/American College of Rheumatology classification criteria for systemic lupus erythematosus","volume":"78","author":"Aringer","year":"2019","journal-title":"Ann. Rheum. Dis."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"102354","DOI":"10.1016\/j.autrev.2019.102354","article-title":"Characteristics and prognosis of IgG4-related periaortitis\/periarteritis: A systematic literature review","volume":"18","author":"Akiyama","year":"2019","journal-title":"Autoimmun. Rev."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1186\/s12872-020-01813-6","article-title":"Coronary artery vasculitis: A review of current literature","volume":"21","author":"Khanna","year":"2021","journal-title":"BMC Cardiovasc. Disord."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1093\/mrcr\/rxad065","article-title":"Solitary recurrence of IgG4-related giant coronary aneurysm: Case report and review of the literature focusing on treatment strategies and complications","volume":"8","author":"Kawahara","year":"2023","journal-title":"Mod. Rheumatol. Case Rep."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1002\/art.41120","article-title":"The 2019 American College of Rheumatology\/European League Against Rheumatism Classification Criteria for IgG4-Related Disease","volume":"72","author":"Wallace","year":"2020","journal-title":"Arthritis Rheumatol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1806","DOI":"10.1161\/CIR.0000000000000205","article-title":"Revision of the Jones Criteria for the Diagnosis of Acute Rheumatic Fever in the Era of Doppler Echocardiography: A Scientific Statement From the American Heart Association","volume":"131","author":"Gewitz","year":"2015","journal-title":"Circulation"},{"key":"ref_16","doi-asserted-by":"crossref","unstructured":"Matteucci, A., Pignalberi, C., Pandozi, C., Magris, B., Meo, A., Russo, M., Galeazzi, M., Schiaffini, G., Aquilani, S., and Di Fusco, S.A. (2024). Prevention and Risk Assessment of Cardiac Device Infections in Clinical Practice. J. Clin. Med., 13.","DOI":"10.3390\/jcm13092707"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"711","DOI":"10.1016\/j.repc.2023.01.023","article-title":"Treatment with gentamicin-impregnated collagen sponges in reducing infection of implantable cardiac devices: 10-year analysis with propensity score matching","volume":"42","author":"Matteucci","year":"2023","journal-title":"Rev. Port. Cardiol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"39411","DOI":"10.1038\/s41598-025-23081-0","article-title":"Argo Delphi consensus statement on red flags and clinical gateways towards rare disease diagnosis","volume":"15","author":"Limongelli","year":"2025","journal-title":"Sci. Rep."}],"container-title":["Life"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-1729\/16\/4\/537\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T10:36:09Z","timestamp":1774434969000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-1729\/16\/4\/537"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,24]]},"references-count":18,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2026,4]]}},"alternative-id":["life16040537"],"URL":"https:\/\/doi.org\/10.3390\/life16040537","relation":{},"ISSN":["2075-1729"],"issn-type":[{"value":"2075-1729","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3,24]]}}}