{"id":7498,"date":"2020-05-14T20:44:47","date_gmt":"2020-05-14T20:44:47","guid":{"rendered":"https:\/\/medicalcriteria.com\/web\/?p=7498"},"modified":"2026-01-08T19:17:48","modified_gmt":"2026-01-08T19:17:48","slug":"hereditary-angioedema","status":"publish","type":"post","link":"https:\/\/medicalcriteria.com\/web\/hereditary-angioedema\/","title":{"rendered":"Diagnosis of Hereditary Angioedema (HAE)"},"content":{"rendered":"<div class=\"99c380e4b4a7b96c35d7ddf7dcb434e8\" data-index=\"1\" style=\"float: none; margin:0px 0 0px 0; text-align:center;\">\n<script async src=\"https:\/\/pagead2.googlesyndication.com\/pagead\/js\/adsbygoogle.js\"><\/script>\r\n<!-- MC 2019- Horizontal -->\r\n<ins class=\"adsbygoogle\"\r\n     style=\"display:block\"\r\n     data-ad-client=\"ca-pub-0127150553352455\"\r\n     data-ad-slot=\"3806776041\"\r\n     data-ad-format=\"auto\"\r\n     data-full-width-responsive=\"true\"><\/ins>\r\n<script>\r\n     (adsbygoogle = window.adsbygoogle || []).push({});\r\n<\/script>\n<\/div>\n<p>Angioedema, also known as Quincke edema or \u201cangioneurotic edema&#8221;, is defined as the localized nonpitting edema of deep dermal, subcutaneous, or submucosal tissues resulting from the increase in vascular permeability and extravasation of intravascular fluids; although it can coincide with urticaria in a histamine-mediated process, a differentiating feature is that urticarial wheals are limited to the mid and papillary dermis.<!--more--><\/p>\n<p><strong>Diagnosis of Hereditary Angioedema with Normal C1 Inhibitor Levels<\/strong><\/p>\n<p><strong>Consensus criteria*<\/strong><\/p>\n<ul>\n<li>History of recurrent angioedema in the absence of concomitant urticaria or<br \/>\nuse of a medication known to cause angioedema<\/li>\n<li>Normal or near-normal C4 level and C1 inhibitor antigen level and function<\/li>\n<li>Documented lack of response to high-dose antihistamines (e.g., second generation antihistamines given 4 times\/day)<\/li>\n<li>Either a known genetic mutation (factor XII, angiopoietin-1, plasminogen,<br \/>\nor kininogen-1) or a family history of angioedema\u2020<\/li>\n<\/ul>\n<p><strong>Supportive data<\/strong><\/p>\n<ul>\n<li>History of no response to epinephrine and glucocorticoids<\/li>\n<li>History of prompt and durable responses to a bradykinin-targeted medication\u2021<\/li>\n<li>Documented, visible angioedema or, in patients with predominantly abdominal symptoms, evidence of bowel-wall edema identified by computed axial<br \/>\ntomography or magnetic resonance imaging\u00a7<\/li>\n<\/ul>\n<p><strong>Emerging biomarkers<\/strong><\/p>\n<ul>\n<li>Threshold-stimulated kallikrein activity<\/li>\n<\/ul>\n<p>* All four criteria must be met. If there is no family history and no biomarker has been determined, compelling supportive data may suggest the diagnosis.<br \/>\n\u2020 Mutational analysis for plasminogen, angiopoietin-1, and kininogen-1 is available only at a limited number of research facilities.<br \/>\n\u2021 Prompt, durable responses are those that occur within 30 to 120 minutes after administration of the medication and that last for more than 6 hours.<br \/>\n\u00a7 Ultrasonography could be used, although it is less sensitive. In the case of negative findings, we recommend computed axial tomography or magnetic resonance imaging.<\/p>\n<p>&nbsp;<\/p>\n<p><strong>References:<\/strong><\/p>\n<ol>\n<li>Busse PJ, Christiansen SC. Hereditary Angioedema. N Engl J Med. 2020;382(12):1136\u20101148. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/32187470\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>Henao MP, Kraschnewski JL, Kelbel T, Craig TJ. Diagnosis and screening of patients with hereditary angioedema in primary care. Ther Clin Risk Manag. 2016;12:701\u2010711. Published 2016 May 2. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/27194914\/\">[Medline]<\/a><\/li>\n<li>Gill P, Betschel SD. The Clinical Evaluation of Angioedema. Immunol Allergy Clin North Am. 2017;37(3):449\u2010466. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/28687102\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<\/ol>\n<p>&nbsp;<\/p>\n<p>Created May 08, 2020.<\/p>\n\n<div style=\"font-size: 0px; height: 0px; line-height: 0px; margin: 0; padding: 0; clear: both;\"><\/div>","protected":false},"excerpt":{"rendered":"<p>Sorry, this entry is only available in Espa\u00f1ol.<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_lmt_disableupdate":"no","_lmt_disable":"no","_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"footnotes":""},"categories":[568],"tags":[2163,123,13,2164,711],"class_list":["post-7498","post","type-post","status-publish","format-standard","hentry","category-allergy","tag-angioedema","tag-diagnosis","tag-diagnostico","tag-hereditario","tag-hereditary"],"modified_by":"Guillermo Firman","_links":{"self":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/7498","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/comments?post=7498"}],"version-history":[{"count":9,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/7498\/revisions"}],"predecessor-version":[{"id":9359,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/7498\/revisions\/9359"}],"wp:attachment":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/media?parent=7498"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/categories?post=7498"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/tags?post=7498"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}