{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,11]],"date-time":"2026-05-11T22:12:36Z","timestamp":1778537556349,"version":"3.51.4"},"reference-count":24,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2026,2,3]],"date-time":"2026-02-03T00:00:00Z","timestamp":1770076800000},"content-version":"vor","delay-in-days":2,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"Rutishauser Travel"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,5,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>Dilated cardiomyopathy (DCM) encompasses genetic and acquired aetiologies, yet the impact of immune-mediated mechanisms remains unclear. This study investigates the clinical characteristics and outcomes of immune-mediated DCM (ID-CMP).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>From the Maastricht Cardiomyopathy registry, 194 patients with ID-CMP were compared with 678 non-immune DCM controls over a median follow-up of 5.2 (3.7\u201310.7) years. ID-CMP was categorized into autoimmune-mediated (adaptive immunity dysfunction), autoinflammatory-driven (innate immunity dysfunction), and mixed-pattern (MHC Class I associations and autoinflammatory components). Patients with features of &amp;gt;1 category were classified as overlapping-CMP. Echocardiographic and genetic data were collected. Cox regression assessed major adverse cardiovascular events (MACE: cardiac mortality, heart failure hospitalization, life-threatening arrhythmias). Left ventricular ejection fraction (LVEF) trajectories were analysed using linear mixed-effects models. Findings were validated in an independent cohort of 201 ID-CMP patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>ID-CMP was associated with a higher risk of MACE [HR: 1.68 (1.1\u20132.5), P = .012] after adjustment for age, sex, and baseline LVEF, confirmed in an external validation cohort [HR:2.40 (1.6\u20133.5), P &amp;lt; .001]. LVEF was lower in ID-CMP during the first year but converged with non-immune DCM thereafter (P0\u20131year = .018; P&amp;gt;1year = ns). Outcomes did not differ among ID-CMP subtypes. Inflammation on EMB was related to worse clinical outcome [HR:2.0 (1.1\u20133.6), P = .017]. Pathogenic DCM gene variants were equally frequent in ID-CMP and non-ID-CMP patients (22% vs 20%, P = ns).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>ID-CMP patients have a higher risk of MACE despite similar LVEF trajectories, as confirmed in a validation cohort. Cardiac immune cell infiltration suggests an inflammatory substrate independent of genetics.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ejhf\/xuaf030","type":"journal-article","created":{"date-parts":[[2026,1,6]],"date-time":"2026-01-06T12:27:10Z","timestamp":1767702430000},"page":"278-287","source":"Crossref","is-referenced-by-count":0,"title":["Clinical characteristics and outcomes of cardiomyopathies related to immune-mediated diseases"],"prefix":"10.1093","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9235-6597","authenticated-orcid":false,"given":"Valentina A","family":"Rossi","sequence":"first","affiliation":[{"name":"Department of Cardiology, Maastricht University, CARIM School for Cardiovascular Diseases ,","place":["The Netherlands"]},{"name":"Department of Cardiology, University Heart Center, University Hospital and University of Zurich , Zurich ,","place":["Switzerland"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8165-6631","authenticated-orcid":false,"given":"Maurits A","family":"Sikking","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Maastricht University, CARIM School for Cardiovascular Diseases ,","place":["The Netherlands"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9012-3439","authenticated-orcid":false,"given":"Alessandro","family":"Folgheraiter","sequence":"additional","affiliation":[{"name":"Cardiovascular Department, Centre for Diagnosis and Treatment of Cardiomyopathies, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste , Trieste ,","place":["Italy"]},{"name":"European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart , Amsterdam ,","place":["The 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Majadahonda and Centro Nacional de Investigaciones Cardiovasculares (CNIC) , Madrid ,","place":["Spain"]},{"name":"CIBER Cardiovascular, Instituto de Salud Carlos III , Madrid ,","place":["Spain"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0956-6479","authenticated-orcid":false,"given":"Bastien S C","family":"Nihant","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Maastricht University, CARIM School for Cardiovascular Diseases ,","place":["The Netherlands"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0965-6359","authenticated-orcid":false,"given":"Sophie L V M","family":"Stroeks","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Maastricht University, CARIM School for Cardiovascular Diseases ,","place":["The Netherlands"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6222-071X","authenticated-orcid":false,"given":"Michiel T H M","family":"Henkens","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Maastricht University, CARIM School for Cardiovascular Diseases ,","place":["The Netherlands"]},{"name":"Netherlands Heart Institute (NLHI), Utrecht, The \u00a0","place":["Netherlands"]},{"name":"Department of Pathology, Maastricht University Medical Centre, Maastricht, The \u00a0","place":["Netherlands"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nerea","family":"Mora-Ayestar\u00e1n","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Hospital Universitario Puerta de Hierro Majadahonda and Centro Nacional de Investigaciones Cardiovasculares (CNIC) , Madrid ,","place":["Spain"]},{"name":"CIBER Cardiovascular, Instituto de Salud Carlos III , Madrid ,","place":["Spain"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Noem\u00ed","family":"Ramos-L\u00f3pez","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Hospital Universitario Puerta de Hierro Majadahonda and Centro Nacional de Investigaciones Cardiovasculares (CNIC) , Madrid ,","place":["Spain"]},{"name":"CIBER Cardiovascular, Instituto de Salud Carlos III , Madrid ,","place":["Spain"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2700-8478","authenticated-orcid":false,"given":"Gianfranco","family":"Sinagra","sequence":"additional","affiliation":[{"name":"Cardiovascular Department, Centre for Diagnosis and Treatment of Cardiomyopathies, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste , Trieste ,","place":["Italy"]},{"name":"European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart , Amsterdam ,","place":["The 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