{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,13]],"date-time":"2025-11-13T07:14:14Z","timestamp":1763018054404,"version":"3.37.3"},"reference-count":26,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2019,9,17]],"date-time":"2019-09-17T00:00:00Z","timestamp":1568678400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"Centre for Biomedical Network Research on Rare Diseases"},{"name":"Complemento II-CM network","award":["B2017\/BMD3673"],"award-info":[{"award-number":["B2017\/BMD3673"]}]},{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III","doi-asserted-by":"publisher","award":["PI15-00255"],"award-info":[{"award-number":["PI15-00255"]}],"id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003329","name":"Ministerio de Econom\u00eda y Competitividad","doi-asserted-by":"publisher","award":["B2017","BMD3673"],"award-info":[{"award-number":["B2017","BMD3673"]}],"id":[{"id":"10.13039\/501100003329","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,11,15]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Acquired angioedema due to C1-inhibitor (C1INH) deficiency (AAE) is caused by secondary C1INH deficiency leading to bradykinin-mediated angioedema episodes. AAE typically presents in adulthood and is associated with B cell lymphoproliferation. Anti-C1INH autoantibodies (antiC1INHAbs) are detectable in a subset of AAE cases and considered a hallmark of the disease. When free antiC1INHAbs and malignant tumors are not detectable, diagnosis relies on the finding of low C1INH levels and\/or function, lack of family history and SERPING1 mutations, age at onset and low or undetectable C1q levels, none of which is specific for AAE. We tested the diagnostic value of a novel enzyme-linked immunosorbent assay (ELISA) for the detection of circulating complexes between C1INH and antiC1INHAbs (C1INH\u2013antiC1INHAb) in the serum of 20 European AAE patients characterized on the basis of their complement levels and function. Free antiC1INHAbs were detected in nine of 20 patients [six of immunoglobulin (Ig)G class, two of IgM class and one simultaneously presenting IgG and IgM classes], whereas C1INH\u2013antiC1INHAb complexes were found in 18 of 20 of the AAE cases, regardless of the presence or absence of detectable free anti-C1INHAbs. Of note, nine of 20 patients showed negative free antiC1INHabs, but positive C1INH\u2013antiC1INHAb complexes in their first measurement. In the cohort presented, IgM-class C1INH\u2013antiC1INHAb are specifically and strongly associated with low C1q serum levels. Detection of C1INH\u2013antiC1-INHAbs provides an added value for AAE diagnosis, especially in those cases in whom no free anti-C1INH antibodies are detected. The link between IgM-class C1INH\u2013antiC1INHAb complexes and C1q consumption could have further implications for the development of autoimmune manifestations in AAE.<\/jats:p>","DOI":"10.1111\/cei.13361","type":"journal-article","created":{"date-parts":[[2019,8,9]],"date-time":"2019-08-09T14:43:35Z","timestamp":1565361815000},"page":"341-350","source":"Crossref","is-referenced-by-count":10,"title":["Serum complexes between C1INH and C1INH autoantibodies for the diagnosis of acquired angioedema"],"prefix":"10.1093","volume":"198","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9596-6910","authenticated-orcid":false,"given":"A","family":"L\u00f3pez-Lera","sequence":"first","affiliation":[{"name":"Instituto de Investigaci\u00f3n Sanitaria del Hospital La Paz (IdiPaz), Madrid, Spain"},{"name":"Centre for Biomedical Network Research on Rare Diseases (CIBERER) U-754, Hospital Universitario La Paz, Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S","family":"Garrido","sequence":"additional","affiliation":[{"name":"Centre for Biomedical Network Research on Rare Diseases (CIBERER) U-754, Hospital Universitario La Paz, Madrid, Spain"},{"name":"Immunology Unit, Hospital Universitario La Paz, Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P","family":"Nozal","sequence":"additional","affiliation":[{"name":"Centre for Biomedical Network Research on Rare Diseases (CIBERER) U-754, Hospital Universitario La Paz, Madrid, Spain"},{"name":"Immunology Unit, Hospital Universitario La Paz, Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L","family":"Skatum","sequence":"additional","affiliation":[{"name":"Clinical Immunology and Transfusion Medicine, Office for Medical Services, Lund, Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A","family":"Bygum","sequence":"additional","affiliation":[{"name":"National HAE Centre, Odense University Hospital, Denmark"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T","family":"Caballero","sequence":"additional","affiliation":[{"name":"Instituto de Investigaci\u00f3n Sanitaria del Hospital La Paz (IdiPaz), Madrid, Spain"},{"name":"Centre for Biomedical Network Research on Rare Diseases (CIBERER) U-754, Hospital Universitario La Paz, Madrid, Spain"},{"name":"Department of Allergy, Hospital Universitario La Paz, Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M","family":"L\u00f3pez Trascasa","sequence":"additional","affiliation":[{"name":"Instituto de Investigaci\u00f3n Sanitaria del Hospital La Paz (IdiPaz), Madrid, Spain"},{"name":"Departamento de Medicina, Universidad Aut\u00f3noma de Madrid, Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2019,9,17]]},"reference":[{"key":"2022011418370184600_cei13361-bib-0001","first-page":"14","article-title":"Acquired angioedema. 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