{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T11:50:55Z","timestamp":1753876255914,"version":"3.41.2"},"reference-count":11,"publisher":"Oxford University Press (OUP)","issue":"9","license":[{"start":{"date-parts":[[2021,8,18]],"date-time":"2021-08-18T00:00:00Z","timestamp":1629244800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,9,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare disease due to small vessel inflammation and characterized by chronic urticarial vasculitis and arthritis. Multi-organ manifestations may include glomerulonephritis, ocular inflammation (uveitis, episcleritis), and recurrent abdominal pain. To the best of our knowledge, just other nine cases of HUVS with cardiac valvular involvement have been reported in the literature.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Case summary<\/jats:title>\n                  <jats:p>A 32-year-old woman presented to the emergency department because of a cerebral haemorrhage. She suffered from a severe HUVS form with cardiac valvular involvement. In the previous years, she underwent cardiac surgery twice for aortic and mitral valves immune-mediated degeneration. The neurologic event was secondary to Listeria monocytogenes aortic endocarditis, complicated by a cerebral embolism and periaortic abscess.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Discussion<\/jats:title>\n                  <jats:p>Patients with HUVS rarely present valvular heart disease. The latter is mostly secondary to an inflammatory process. Valve degeneration and immunosuppressive therapy increase the risk of infective endocarditis, with dramatic consequences for the prognosis of these patients. Valvular involvement is a sporadic but potentially fatal complication of HUVS, which should be taken in mind in the multidisciplinary evaluation of these patients.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytab341","type":"journal-article","created":{"date-parts":[[2021,8,18]],"date-time":"2021-08-18T15:44:09Z","timestamp":1629301449000},"source":"Crossref","is-referenced-by-count":4,"title":["Heart valve disease in hypocomplementemic urticarial vasculitis syndrome: from immune-mediated degeneration to embolic complications of infective endocarditis\u2014a case report"],"prefix":"10.1093","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8182-7074","authenticated-orcid":false,"given":"Valentina","family":"Scheggi","sequence":"first","affiliation":[{"name":"Division of Cardiovascular and Perioperative Medicine, Cardiothoracovascular Department, Clinical and Experimental Medicine, Azienda Ospedaliero-Universitaria Careggi, University of Florence, Largo Brambilla 3, 50134 Florence, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8101-4695","authenticated-orcid":false,"given":"Niccol\u00f2","family":"Marchionni","sequence":"additional","affiliation":[{"name":"Division of General Cardiology, Cardiothoracovascular Department, Clinical and Experimental Medicine, Azienda Ospedaliero-Universitaria Careggi, University of Florence, Largo Brambilla 3, 50134 Florence, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pier Luigi","family":"Stef\u00e0no","sequence":"additional","affiliation":[{"name":"Division of Cardiac Surgery, Cardiothoracovascular Department, Clinical and Experimental Medicine, Azienda Ospedaliero-Universitaria Careggi, University of Florence, Largo Brambilla 3, 50134 Florence, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2021,8,18]]},"reference":[{"key":"2021091809013375800_ytab341-B1","doi-asserted-by":"crossref","first-page":"837","DOI":"10.1177\/0961203308090113","article-title":"Hypocomplementemic urticarial vasculitis with Jaccoud's arthropathy and valvular heart disease: case report and review of the literature","volume":"17","author":"Amano","year":"2008","journal-title":"Lupus"},{"key":"2021091809013375800_ytab341-B2","doi-asserted-by":"crossref","first-page":"352","DOI":"10.1186\/1752-1947-8-352","article-title":"Membranoproliferative glomerulonephritis presenting as arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis: a case report","volume":"8","author":"Park","year":"2014","journal-title":"J Med Case Rep"},{"key":"2021091809013375800_ytab341-B3","first-page":"383","article-title":"Hypocomplementemic urticarial vasculitis, Jaccoud's arthropathy, valvular heart disease, and reversible tracheal stenosis: a surfeit of syndromes","volume":"28","author":"Chen","year":"2001","journal-title":"J Rheumatol"},{"key":"2021091809013375800_ytab341-B4","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1016\/S1054-8807(02)00107-2","article-title":"Valvular heart disease in patients with hypocomplementemic urticarial vasculitis syndrome associated with Jaccoud's arthropathy","volume":"11","author":"Houser","year":"2002","journal-title":"Cardiovasc Pathol"},{"key":"2021091809013375800_ytab341-B5","doi-asserted-by":"crossref","first-page":"1850","DOI":"10.1111\/1756-185X.12360","article-title":"A case of hypocomplementaemic urticarial vasculitis with cardiac valve involvement successfully treated with cyclophosphamide and high-dose glucocorticoids","volume":"20","author":"Hauser","year":"2017","journal-title":"Int J Rheum Dis"},{"key":"2021091809013375800_ytab341-B6","first-page":"1236","article-title":"Hypocomplementemic urticarial vasculitis syndrome, Jaccoud's syndrome, valvulopathy: a new syndromic combination","volume":"20","author":"Palazzo","year":"1993","journal-title":"J Rheumatol"},{"key":"2021091809013375800_ytab341-B7","doi-asserted-by":"crossref","first-page":"1196","DOI":"10.1067\/mai.2000.110805","article-title":"Atypical fatal hypocomplementemic urticarial vasculitis with involvement of native and homograft aortic valves in an African American man","volume":"106","author":"Hong","year":"2000","journal-title":"J Allergy Clin Immunol"},{"key":"2021091809013375800_ytab341-B8","doi-asserted-by":"crossref","first-page":"3075","DOI":"10.1093\/eurheartj\/ehv319","article-title":"2015 ESC Guidelines for the management of infective endocarditis: the Task Force for the Management of Infective Endocarditis of the European Society of Cardiology (ESC). 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