{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T19:32:36Z","timestamp":1779910356132,"version":"3.53.1"},"reference-count":0,"publisher":"S. Karger AG","issue":"5","license":[{"start":{"date-parts":[[2022,1,1]],"date-time":"2022-01-01T00:00:00Z","timestamp":1640995200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2022,1,1]],"date-time":"2022-01-01T00:00:00Z","timestamp":1640995200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Int Arch Allergy Immunol"],"published-print":{"date-parts":[[2022]]},"abstract":"<b><i>Background:<\/i><\/b> Acquired deficiency of C1 inhibitor (AAE-C1-INH) is a very rare cause of recurrent angioedema, with few cases reported in the literature. We aimed to describe a series of patients with AAE-C1-INH who were diagnosed and received care at angioedema reference centers in Brazil, affiliated to the Brazilian Group of Studies on Hereditary Angioedema. <b><i>Methods:<\/i><\/b> Fourteen patients from 8 Brazilian Angioedema Reference Centers, diagnosed with AAE-C1-INH, were included in this study. Clinical data collected included sex, date of birth, date of onset of symptoms, date of diagnosis, plasma levels of antigenic and\/or functional C1-INH, levels of C4 and C1q, location and treatment of angioedema attacks, long-term prophylaxis, associated diseases, and definitive treatment. <b><i>Results:<\/i><\/b> Fourteen patients were identified with AAE-C1-INH. Most patients (10\/14; 71.4%) were female. The median age at onset of symptoms was 56.5 years (range, 14\u201374 years; interquartile range [IQR], 32\u201364 years), and median age at diagnosis was 58.0 years (range, 20\u201376 years; IQR, 38\u201365 years), with a median time until diagnosis of 2 years (range, 0\u20136 years; IQR, 1\u20133 years). The most common manifestations were cutaneous (face, eyelids, lips, trunk, hands, feet, and genitals). Most patient had low levels of C4 (13\/14; 92.8%) and of antigenic C1-INH (8\/14; 57.1%). Four had decreased functional activity of C1-INH (4\/7; 57.1%) and C1q levels were low in 5 patients (5\/12; 41.6%). Underlying diseases were identified in all 14 patients, with lymphoma of the splenic marginal zone and monoclonal gammopathy of undetermined significance being the most frequent. Nine patients (64.2%) needed long-term prophylactic treatment for recurrent angioedema and 5 patients (46.7%) required treatment for angioedema attacks. Most of them (12\/14; 85.7%) had resolution of angioedema. <b><i>Conclusion:<\/i><\/b> Therapy of AAE-C1-INH aims to control symptoms; however, diagnosis and treatment of the underlying disease, when present, should be an important target and may lead to the resolution of angioedema in patients with AAE-C1-INH. <\/jats:p>","DOI":"10.1159\/000521646","type":"journal-article","created":{"date-parts":[[2022,3,24]],"date-time":"2022-03-24T22:01:23Z","timestamp":1648159283000},"page":"572-577","source":"Crossref","is-referenced-by-count":4,"title":["Acquired Angioedema due to C1-Inhibitor Deficiency: A Challenging Condition"],"prefix":"10.1159","volume":"183","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5512-7349","authenticated-orcid":false,"given":"Solange Oliveira Rodrigues","family":"Valle","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7879-7134","authenticated-orcid":false,"given":"Maria Luiza Oliva","family":"Alonso","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0188-4656","authenticated-orcid":false,"given":"S\u00e9rgio Duarte","family":"Dortas Junior","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ekaterini Sim\u00f5es","family":"Goudouris","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Luiza Ribeiro Bard","family":"de Carvalho","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Albertina Varandas","family":"Capelo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6450-6930","authenticated-orcid":false,"given":"Eli","family":"Mansour","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Fl\u00e1via","family":"Bernardes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5581-0444","authenticated-orcid":false,"given":"Luiz Fernando Bacarini","family":"Leite","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1034-7580","authenticated-orcid":false,"given":"Pedro","family":"Giavina-Bianchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcelo Vivolo","family":"Aun","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariana Paes Leme","family":"Ferriani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luisa Karla","family":"Arruda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9803-0309","authenticated-orcid":false,"given":"Anete Sevciovic","family":"Grumach","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2022,3,24]]},"container-title":["International Archives of Allergy and Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/521646","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/521646","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T15:42:36Z","timestamp":1745422956000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000521646"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022]]},"references-count":0,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2022,5,2]]},"published-print":{"date-parts":[[2022,5,3]]}},"URL":"https:\/\/doi.org\/10.1159\/000521646","archive":["Portico"],"relation":{},"ISSN":["1018-2438","1423-0097"],"issn-type":[{"value":"1018-2438","type":"print"},{"value":"1423-0097","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022]]}}}