Hereditary angioedema: how to approach it at the emergency department?

Abstract

Angioedema attacks are common causes of emergency care, and due to the potential for severity, it is important that professionals who work in these services know their causes and management. The mechanisms involved in angioedema without urticaria may be histamine- or bradykinin-mediated. The most common causes of histamine-mediated angioedema are foods, medications, insect sting and idiopathic. When the mediator is bradykinin, the triggers are angiotensin-converting enzyme inhibitors and factors related to acquired angioedema with deficiency of C1-inhibitor or hereditary angioedema, which are less common, but very important because of the possibility of fatal outcome. Hereditary angioedema is a rare disease characterized by attacks of edema that affect the subcutaneous tissue and mucous membranes of various organs, manifesting mainly by angioedema and abdominal pain. This type of angioedema does not respond to the usual treatment with epinephrine, antihistamines and corticosteroids. Thus, if not identified and treated appropriately, these patients have an estimated risk of mortality from laryngeal edema of 25% to 40%. Hereditary angioedema treatment has changed dramatically in recent years with the development of new and efficient drugs for attack management: plasma-derived C1 inhibitor, recombinant human C1-inhibitor, bradykinin B2 receptor antagonist (icatibant), and the kallikrein inhibitor (ecallantide). In Brazil, plasma-derived C1 inhibitor and icatibant have already been approved for use. Proper management of these patients in the emergency department avoids unnecessary surgery and, especially, fatal outcomes.

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2021 revision and update

“…177,343 Emergency departments and other medical treatment facilities that provide acute care are strongly advised to develop and implement angioedema management algorithms and train their staff to effectively recognize and treat laryngeal and abdominal HAE attacks. [357][358][359][360]…”

The international WAO/EAACI guideline for the management of hereditary angioedema – The 2021 revision and update

“…Emergency departments and other medical treatment facilities that provide acute care are strongly advised to develop and implement angioedema management algorithms and train their staff to effectively recognize and treat laryngeal and abdominal HAE attacks. 357 , 358 , 359 , 360 …”

Perfil clínico de pacientes com Angioedema Hereditário atendidos em um hospital escola da Cidade de Maceió, Alagoas

“…O Angioedema Hereditário (AEH) é um edema não inflamatório, assimétrico e de origem autossômica dominante, atingindo um em cada 50.000 indivíduos. Caracterizada por ser autolimitada, esta doença restringe-se ao tecido subcutâneo ou submucoso e ocorre devido ao aumento da permeabilidade vascular e da vasodilatação, causando o inchaço da região atingida (Giavina-Bianchi et al, 2018;Serpa et al, 2021;Arruda & Leme Ferriani, 2021). Os episódios de AEH são recorrentes, devido a mutação no gene SERPING1 que decorre em função da deficiência do inibidor de C1 (C1-INH), induzindo a produção, em excesso, do mediador vasoativo bradicinina, de modo a afetar diversos órgãos, sendo que um terço das crises se localizam na região abdominal e outro terço na região dos membros (Busse & Christiansen, 2020;.…”

“…Os episódios de AEH são recorrentes, devido a mutação no gene SERPING1 que decorre em função da deficiência do inibidor de C1 (C1-INH), induzindo a produção, em excesso, do mediador vasoativo bradicinina, de modo a afetar diversos órgãos, sendo que um terço das crises se localizam na região abdominal e outro terço na região dos membros (Busse & Christiansen, 2020;. Demais sítios, segundo Aygören-Pürsün e , como rosto, pescoço e genitais, também podem ser afetados, no entanto, ressalta-se que as regiões com manifestações clínicas mais graves se relacionam com as vias aéreas superiores, pois podem causar morte por asfixia, quando a laringe é acometida (Bork, Hardt & Witzke, 2012;Johnson e Phillips, 2018;Serpa et al, 2021).…”