{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T16:05:49Z","timestamp":1791993949403,"version":"4.4.0"},"reference-count":28,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"17","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2018,9,4]]},"abstract":"<jats:sec xml:lang=\"en\">\n            <jats:title>Background<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              Glycosphingolipid accumulation in Fabry cells generates a proinflammatory response that may influence disease evolution and responsiveness to enzyme replacement therapy. This study evaluated incidence, mechanism, and impact of myocarditis in Fabry disease cardiomyopathy (\n              <jats:styled-content style=\"fixed-case\">FDCM<\/jats:styled-content>\n              ).\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Methods and Results<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              Myocarditis, defined as\n              <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>\n              3\n              <jats:sup>+<\/jats:sup>\n              T lymphocytes &gt;7\/mm\n              <jats:sup>2<\/jats:sup>\n              associated with necrosis of glycolipid\u2010laden myocardiocytes, was retrospectively evaluated in endomyocardial biopsies from 78 patients with\n              <jats:styled-content style=\"fixed-case\">FDCM<\/jats:styled-content>\n              : 13 with maximal wall thickness (MWT) &lt;11\u00a0mm (group 1), 17 with MWT 11 to 15\u00a0mm (group 2), 30 with MWT 16 to 20\u00a0mm (group 3), and 18 with MWT &gt;20\u00a0mm (group 4). Myocarditis was investigated by polymerase chain reaction for cardiotropic viruses, by serum antiheart and antimyosin antibodies, and by cardiac magnetic resonance. Myocarditis was recognized at histology in 48 of 78 patients with\n              <jats:styled-content style=\"fixed-case\">FDCM<\/jats:styled-content>\n              (38% of group 1, 41% of group 2, 66% of group 3, and 72% of group 4). Myocarditis was characterized by positive antiheart and antimyosin antibodies and negative polymerase chain reaction for viral genomes.\n              <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>\n              3\n              <jats:sup>+<\/jats:sup>\n              cells\/mm\n              <jats:sup>2<\/jats:sup>\n              correlated with myocyte necrosis, antimyosin autoantibody titer, and\n              <jats:styled-content style=\"fixed-case\">MWT<\/jats:styled-content>\n              (\n              <jats:italic>P<\/jats:italic>\n              &lt;0.001,\n              <jats:italic>r<\/jats:italic>\n              =0.79;\n              <jats:italic>P<\/jats:italic>\n              &lt;0.001,\n              <jats:italic>r<\/jats:italic>\n              =0.84;\n              <jats:italic>P<\/jats:italic>\n              &lt;0.001,\n              <jats:italic>r<\/jats:italic>\n              =0.61, respectively). Cardiac magnetic resonance showed myocardial edema in 24 of 78 patients (31%): 0% of group 1, 23% of group 2, 37% of group 3, and 50% of group 4.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              Myocarditis is detectable at histology in up to 56% of patients with\n              <jats:styled-content style=\"fixed-case\">FDCM<\/jats:styled-content>\n              . It is immune mediated and correlates with disease severity. It can be disclosed by antiheart\/antimyosin autoantibodies and in the advanced phase by cardiac magnetic resonance. It may contribute to progression of\n              <jats:styled-content style=\"fixed-case\">FDCM<\/jats:styled-content>\n              and resistance to enzyme replacement therapy.\n            <\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/jaha.118.009052","type":"journal-article","created":{"date-parts":[[2018,9,17]],"date-time":"2018-09-17T18:20:49Z","timestamp":1537208449000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":67,"title":["Immune\u2010Mediated Myocarditis in Fabry Disease Cardiomyopathy"],"prefix":"10.1161","volume":"7","author":[{"given":"Andrea","family":"Frustaci","sequence":"first","affiliation":[{"name":"Department of Cardiovascular Nephrologic, Anesthetic and Geriatric Sciences La Sapienza University of Rome Italy"},{"name":"Molecular and Cellular Cardiology Lab IRCCS \u2018L. Spallanzani\u2019 Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Romina","family":"Verardo","sequence":"additional","affiliation":[{"name":"Molecular and Cellular Cardiology Lab IRCCS \u2018L. Spallanzani\u2019 Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"Grande","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Nephrologic, Anesthetic and Geriatric Sciences La Sapienza University of Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicola","family":"Galea","sequence":"additional","affiliation":[{"name":"Department of Radiological, Oncological and Pathological Sciences Sapienza University Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierluca","family":"Piselli","sequence":"additional","affiliation":[{"name":"Department of Epidemiology and Preclinical Research IRCCS \u2018L. Spallanzani\u2019 Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iacopo","family":"Carbone","sequence":"additional","affiliation":[{"name":"Department of Radiological, Oncological and Pathological Sciences Sapienza University Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Alfarano","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Nephrologic, Anesthetic and Geriatric Sciences La Sapienza University of Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matteo Antonio","family":"Russo","sequence":"additional","affiliation":[{"name":"MEBIC Consortium San Raffaele 21 University Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Chimenti","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Nephrologic, Anesthetic and Geriatric Sciences La Sapienza University of Rome Italy"},{"name":"Molecular and Cellular Cardiology Lab IRCCS \u2018L. Spallanzani\u2019 Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_1_2_2","first-page":"3733","volume-title":"The metabolic and molecular bases of inherited disease","author":"Desnick RJ","year":"2001","unstructured":"Desnick RJ, Ioannou YA, Eng CM. Alpha\u2010galactosidase a deficiency: Fabry disease. In: Scriver CR, Beaudet Al, Sly WS, Valle D, eds. The metabolic and molecular bases of inherited disease. New York, NY: McGraw\u2010Hall; 2001:3733\u20133774."},{"key":"e_1_3_1_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-12-449851-8.00035-8"},{"key":"e_1_3_1_4_2","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-5-30"},{"key":"e_1_3_1_5_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.108.841494"},{"key":"e_1_3_1_6_2","doi-asserted-by":"publisher","DOI":"10.1038\/ki.2010.484"},{"key":"e_1_3_1_7_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCHEARTFAILURE.108.769729"},{"key":"e_1_3_1_8_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199102073240607"},{"key":"e_1_3_1_9_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.97.1.365"},{"key":"e_1_3_1_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.10497"},{"key":"e_1_3_1_11_2","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2012030316"},{"key":"e_1_3_1_12_2","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-8-47"},{"key":"e_1_3_1_13_2","doi-asserted-by":"publisher","DOI":"10.1111\/joim.12077"},{"key":"e_1_3_1_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2005.11.070"},{"key":"e_1_3_1_15_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.93.5.879"},{"key":"e_1_3_1_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2009.02.007"},{"key":"e_1_3_1_17_2","doi-asserted-by":"publisher","DOI":"10.1186\/1532-429X-14-90"},{"key":"e_1_3_1_18_2","doi-asserted-by":"publisher","DOI":"10.1186\/1532-429X-15-92"},{"key":"e_1_3_1_19_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.0000048147.15962.31"},{"key":"e_1_3_1_20_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/16.suppl_O.68"},{"key":"e_1_3_1_21_2","first-page":"1","article-title":"Innate and adaptive immune response in Fabry disease","volume":"22","author":"Mauhin W","year":"2015","unstructured":"Mauhin W, Lidove O, Masat E, Mingozzi F, Mariampillai K, Ziza JM, Benveniste O. 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