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

Maurer, Marcus; Magerl, Markus; Betschel, Stephen; Aberer, Werner; Ansotegui, Ignacio J; Aygören-Pürsün, Emel; Banerji, Aleena; Bara, Noémi-Anna; Boccon-Gibod, Isabelle; Bork, Konrad; Bouillet, Laurence; Boysen, Henrik Balle; Brodszki, Nicholas; Busse, Paula J; Bygum, Anette; Caballero, Teresa; Cancian, Mauro; Castaldo, Anthony J; Cohn, Danny M; Csuka, Dorottya; ... (2022). The international WAO/EAACI guideline for the management of hereditary angioedema - The 2021 revision and update. World Allergy Organization journal, 15(3), p. 100627. Wolters Kluwer Health Lippincott Williams & Wilkins 10.1016/j.waojou.2022.100627

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Hereditary Angioedema (HAE) is a rare and disabling disease for which early diagnosis and effective therapy are critical. This revision and update of the global WAO/EAACI guideline on the diagnosis and management of HAE provides up-to-date guidance for the management of HAE. For this update and revision of the guideline, an international panel of experts reviewed the existing evidence, developed 28 recommendations, and established consensus by an online DELPHI process. The goal of these recommendations and guideline is to help physicians and their patients in making rational decisions in the management of HAE with deficient C1-inhibitor (type 1) and HAE with dysfunctional C1-inhibitor (type 2), by providing guidance on common and important clinical issues, such as: 1) How should HAE be diagnosed? 2) When should HAE patients receive prophylactic on top of on-demand treatment and what treatments should be used? 3) What are the goals of treatment? 4) Should HAE management be different for special HAE patient groups such as children or pregnant/breast feeding women? 5) How should HAE patients monitor their disease activity, impact, and control? It is also the intention of this guideline to help establish global standards for the management of HAE and to encourage and facilitate the use of recommended diagnostics and therapies for all patients.

Item Type:

Journal Article (Review Article)


04 Faculty of Medicine > Pre-clinic Human Medicine > Institute of Pharmacology

UniBE Contributor:

Späth, Peter Julius


600 Technology > 610 Medicine & health




Wolters Kluwer Health Lippincott Williams & Wilkins




Pubmed Import

Date Deposited:

03 May 2022 10:31

Last Modified:

17 May 2023 09:51

Publisher DOI:


PubMed ID:


Uncontrolled Keywords:

C1-inhibitor DELPHI GRADE therapy Hereditary angioedema diagnosis disease control guideline management prophylaxis quality of life recommendations self-administration




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