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

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

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 revi...

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Bibliographic Details
Published in:Allergy (Copenhagen) Vol. 77; no. 7; pp. 1961 - 1990
Main Authors: 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, Cohn, Danny M., Csuka, Dorottya, Farkas, Henriette, Gompels, Mark, Gower, Richard, Grumach, Anete S., Guidos‐Fogelbach, Guillermo, Hide, Michihiro, Kang, Hye‐Ryun, Kaplan, Allen Phillip, Katelaris, Constance, Kiani‐Alikhan, Sorena, Lei, Wei‐Te, Lockey, Richard, Longhurst, Hilary, Lumry, William R., MacGinnitie, Andrew, Malbran, Alejandro, Martinez Saguer, Inmaculada, Matta, Juan José, Nast, Alexander, Nguyen, Dinh, Nieto‐Martinez, Sandra A., Pawankar, Ruby, Peter, Jonathan, Porebski, Grzegorz, Prior, Nieves, Reshef, Avner, Riedl, Marc, Ritchie, Bruce, Rafique Sheikh, Farrukh, Smith, William B., Spaeth, Peter J., Stobiecki, Marcin, Toubi, Elias, Varga, Lilian Agnes, Weller, Karsten, Zanichelli, Andrea, Zhi, Yuxiang, Zuraw, Bruce, Craig, Timothy
Format: Journal Article
Language:English
Published: Denmark Blackwell Publishing Ltd 01-07-2022
Subjects:
Angioedema
Breast
C1 inhibitor
Clinical decision making
Complement component C1
DELPHI
Diagnosis
disease control
Edema
Genetic disorders
guideline
hereditary angioedema
Inflammatory diseases
Management
Medical treatment
Patients
Skin
C1 inhibitor
disease control
hereditary angioedema
management
guideline
DELPHI
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Description
Summary: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.
Bibliography:Funding information
LEN/HAEi network of Angioedema Centers of Reference and Excellence (ACARE
2
Funding and support of the development of this update and revision of the guideline came from WAO and EAACI. This revision and update of the guideline benefitted from the help and support of the GA
Important
As this is an international guideline, no information is provided regarding the licensing of the drugs mentioned for the treatment of HAE. It is in the duty of the treating physician to adhere to the relevant local regulations.
)
Marcus Maurer, Markus Magerl, Stephen Betschel and Timothy Craig contributed equally.
https://acare‐network.com
ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
ObjectType-Instructional Material/Guideline-3
content type line 23
ISSN:0105-4538
1398-9995
DOI:10.1111/all.15214