{"id":22609,"date":"2025-01-22T11:42:56","date_gmt":"2025-01-22T11:42:56","guid":{"rendered":"https:\/\/apps.nhslothian.scot\/refhelp\/?page_id=22609"},"modified":"2025-02-06T13:10:32","modified_gmt":"2025-02-06T13:10:32","slug":"complement-testing-and-angioedema","status":"publish","type":"page","link":"https:\/\/apps.nhslothian.scot\/refhelp\/guidelines\/immunology\/complement-testing-and-angioedema\/","title":{"rendered":"Complement testing and Angioedema"},"content":{"rendered":"\n<p><strong>C1 Esterase<\/strong><strong> Inhibitor (C1-INH) deficiency <\/strong>is associated with Hereditary Angioedema (HAE) and Acquired Angioedema.<\/p>\n\n\n\n<h3 class=\"wp-block-heading\">Hereditary Angioedema (HAE)<\/h3>\n\n\n\n<p><strong><u>HAE Clinical Features<\/u><\/strong><\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Usually starts in&nbsp;<strong>childhood<\/strong>&nbsp;(classically symptoms start at or around puberty)<\/li>\n\n\n\n<li><strong>Recurrent episodes<\/strong>&nbsp;of angioedema and\/or abdominal pain<\/li>\n\n\n\n<li><strong>Urticaria is NOT&nbsp;<\/strong>usually associated with C1 inhibitor deficiency<\/li>\n\n\n\n<li>May involve the larynx therefore can be fatal<\/li>\n\n\n\n<li>Diagnosis is suggested by recurrent episodes of non-urticarial swelling unresponsive to antihistamines, positive family history (autosomal dominant inheritance), episode of laryngeal oedema, recurrent episodes of unexplained abdominal pain and vomiting.<\/li>\n\n\n\n<li>Steroids and antihistamines are not effective in attacks.<\/li>\n\n\n\n<li>Consider if angioedema episodes are drug induced (ACEI); allergic; acquired; idiopathic.<\/li>\n<\/ul>\n\n\n\n<p><strong><u>HAE Investigation<\/u><\/strong><\/p>\n\n\n\n<p>This involves measuring blood Complement C3 &amp; C4 levels, C1- inhibitor levels and C1 inhibitor functional analysis. Complement consumption in vitro can lead to artefactually \u2018low\u2019 complement \/ complement functional levels.<\/p>\n\n\n\n<p><em>Ideally serum samples should reach the laboratory within 4 hours of the sample being taken. Phoning the Immunology laboratory to alert reception is helpful, so that samples can be looked out for and processed appropriately. &nbsp;<\/em><\/p>\n\n\n\n<p>Results of C1 inhibitor level \/ functional activity dictate subtype &#8211; HAE type I, II, or III<\/p>\n\n\n\n<p>If hereditary angioedema (HAE) is diagnosed, patients may need C1 inhibitor concentrate \/ bradykinin receptor antagonists for treating future episodes and should be referred. Acute treatment with plasma derived C1-inhibitor concentrate or recombinant C1 inhibitor shortens attacks.<\/p>\n\n\n\n<p><strong>Complement C3<\/strong>\u00a0<strong>and C4 level<\/strong> measurement can be used to monitor disease activity in SLE. Levels fall with disease activity due to complement consumption.<\/p>\n\n\n\n<p><strong>C.M. &amp; C.C. 22-01-25<\/strong><\/p>\n\n\n\n<div class=\"wp-block-getwid-tabs\" data-active-tab=\"0\"><ul class=\"wp-block-getwid-tabs__nav-links\"><\/ul>\n<div class=\"wp-block-getwid-tabs__nav-link\"><span class=\"wp-block-getwid-tabs__title-wrapper\"><a href=\"#\"><span class=\"wp-block-getwid-tabs__title\">Referral Guidelines<\/span><\/a><\/span><\/div><div class=\"wp-block-getwid-tabs__tab-content-wrapper\"><div class=\"wp-block-getwid-tabs__tab-content\">\n<h4 class=\"wp-block-heading\">Who to refer:<\/h4>\n\n\n\n<p>Patients with HAE\/ C1 inhibitor deficiency should be managed with specialist Immunologist input.<br>Please refer any patients with confirmed \/ suspected C1 inhibitor Deficiency to the Clinical Immunology Primary Immune Deficiency Service based at the Royal Infirmary of Edinburgh \u2013 please see <a href=\"https:\/\/apps.nhslothian.scot\/refhelp\/guidelines\/immunology\/\" target=\"_blank\" rel=\"noreferrer noopener\">Immunology<\/a> for full contact details.<\/p>\n<\/div><\/div>\n\n\n\n<div class=\"wp-block-getwid-tabs__nav-link\"><span class=\"wp-block-getwid-tabs__title-wrapper\"><a href=\"#\"><span class=\"wp-block-getwid-tabs__title\">Primary Care Management<\/span><\/a><\/span><\/div><div class=\"wp-block-getwid-tabs__tab-content-wrapper\"><div class=\"wp-block-getwid-tabs__tab-content\">\n<p><\/p>\n<\/div><\/div>\n\n\n\n<div class=\"wp-block-getwid-tabs__nav-link\"><span class=\"wp-block-getwid-tabs__title-wrapper\"><a href=\"#\"><span class=\"wp-block-getwid-tabs__title\">Resources and Links<\/span><\/a><\/span><\/div><div class=\"wp-block-getwid-tabs__tab-content-wrapper\"><div class=\"wp-block-getwid-tabs__tab-content\">\n<p><\/p>\n<\/div><\/div>\n<\/div>\n","protected":false},"excerpt":{"rendered":"<p>C1 Esterase Inhibitor (C1-INH) deficiency is associated with Hereditary Angioedema (HAE) and Acquired Angioedema. Hereditary Angioedema (HAE) HAE Clinical Features HAE Investigation This involves measuring blood Complement C3 &amp; C4 levels, C1- inhibitor levels and C1 inhibitor functional analysis. Complement consumption in vitro can lead to artefactually &lsquo;low&rsquo; complement \/ complement functional levels. Ideally serum<\/p>\n","protected":false},"author":6,"featured_media":0,"parent":4037,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"categories":[1214],"class_list":["post-22609","page","type-page","status-publish","hentry","category-https-apps-nhslothian-scot-refhelp-complement-testing-and-angioedema"],"publishpress_future_workflow_manual_trigger":{"enabledWorkflows":[]},"rttpg_featured_image_url":null,"rttpg_author":{"display_name":"heatherlevy","author_link":"https:\/\/apps.nhslothian.scot\/refhelp\/author\/heatherlevy\/"},"rttpg_comment":0,"rttpg_category":" <a href=\"https:\/\/apps.nhslothian.scot\/refhelp\/category\/laboratoryservices\/immunology\/https-apps-nhslothian-scot-refhelp-complement-testing-and-angioedema\/\" rel=\"tag\">Complement testing and Angioedema<\/a>","rttpg_excerpt":"C1 Esterase Inhibitor (C1-INH) deficiency is associated with Hereditary Angioedema (HAE) and Acquired Angioedema. Hereditary Angioedema (HAE) HAE Clinical Features HAE Investigation This involves measuring blood Complement C3 &amp; C4 levels, C1- inhibitor levels and C1 inhibitor functional analysis. Complement consumption in vitro can lead to artefactually &lsquo;low&rsquo; complement \/ complement functional levels. Ideally serum","_links":{"self":[{"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/pages\/22609","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/users\/6"}],"replies":[{"embeddable":true,"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/comments?post=22609"}],"version-history":[{"count":4,"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/pages\/22609\/revisions"}],"predecessor-version":[{"id":22689,"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/pages\/22609\/revisions\/22689"}],"up":[{"embeddable":true,"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/pages\/4037"}],"wp:attachment":[{"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/media?parent=22609"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/categories?post=22609"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}