The international WAO/EAACI guideline for the management of hereditary angioedema—The 2021 revision and update
Creators: name: Maurer, Marcus
name: Magerl, Markus
name: Betschel, Stephen
name: Aberer, Werner
name: Ansotegui, Ignacio J.
name: Aygören‐Pürsün, Emel
name: Banerji, Aleena
name: Bara, Noémi‐Anna
name: Boccon‐Gibod, Isabelle
name: Bork, Konrad
name: Bouillet, Laurence
name: Boysen, Henrik Balle
name: Brodszki, Nicholas
name: Busse, Paula J.
name: Bygum, Anette
name: Caballero, Teresa
name: Cancian, Mauro
name: Castaldo, Anthony
name: Cohn, Danny M.
name: Csuka, Dorottya
name: Farkas, Henriette
name: Gompels, Mark
name: Gower, Richard
name: Grumach, Anete S.
name: Guidos‐Fogelbach, Guillermo
name: Hide, Michihiro
name: Kang, Hye‐Ryun
name: Kaplan, Allen Phillip
name: Katelaris, Constance
name: Kiani‐Alikhan, Sorena
name: Lei, Wei‐Te
name: Lockey, Richard
name: Longhurst, Hilary
name: Lumry, William R.
name: MacGinnitie, Andrew
name: Malbran, Alejandro
name: Martinez Saguer, Inmaculada
name: Matta, Juan José
name: Nast, Alexander
name: Nguyen, Dinh
name: Nieto‐Martinez, Sandra A.
name: Pawankar, Ruby
name: Peter, Jonathan
name: Porebski, Grzegorz
name: Prior, Nieves
name: Reshef, Avner
name: Riedl, Marc
name: Ritchie, Bruce
name: Rafique Sheikh, Farrukh
name: Smith, William B.
name: Spaeth, Peter J.
name: Stobiecki, Marcin
name: Toubi, Elias
name: Varga, Lilian Agnes
name: Weller, Karsten
name: Zanichelli, Andrea
name: Zhi, Yuxiang
name: Zuraw, Bruce
name: Craig, Timothy
Publisher: name: Charité - Universitätsmedizin Berlin
Year: 2022
Subjects: subject: C1 inhibitor
subject: DELPHI
subject: disease control
subject: guideline
subject: hereditary angioedema
subject: management
subject: 600 Technik, Medizin, angewandte Wissenschaften::610 Medizin und Gesundheit::610 Medizin und Gesundheit

description: 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? and (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.