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

Marcus Maurer, Markus Magerl, Stephen Betschel, Werner Aberer, Ignacio J. Ansotegui, Emel Aygören-Pürsün, Aleena Banerji, Noémi Anna Bara, Isabelle Boccon-Gibod, Konrad Bork, Laurence Bouillet, Henrik Balle Boysen, Nicholas Brodszki, Paula J. Busse, Anette Bygum, Teresa Caballero, Mauro Cancian, Anthony J. Castaldo, Danny M. Cohn, Dorottya CsukaHenriette Farkas, Mark Gompels, Richard Gower, Anete S. Grumach, Guillermo Guidos-Fogelbach, Michihiro Hide, Hye Ryun Kang, Allen P. Kaplan, Constance H. Katelaris, Sorena Kiani-Alikhan, Wei Te Lei, Richard F. Lockey, Hilary Longhurst, William Lumry, Andrew MacGinnitie, Alejandro Malbran, Inmaculada Martinez Saguer, Juan José Matta Campos, Alexander Nast, Dinh Nguyen, Sandra A. Nieto-Martinez, Ruby Pawankar, Jonathan Peter, Grzegorz Porebski, Nieves Prior, Avner Reshef, Marc Riedl, Bruce Ritchie, Farrukh Rafique Sheikh, William B. Smith, Peter J. Spaeth, Marcin Stobiecki, Elias Toubi, Lilian Agnes Varga, Karsten Weller, Andrea Zanichelli, Yuxiang Zhi, Bruce Zuraw, Timothy Craig

Research output: Contribution to journalArticlepeer-review

49 Scopus citations


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.

Original languageEnglish
Article number100627
JournalWorld Allergy Organization Journal
Issue number3
StatePublished - Mar 2022


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


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