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 Castaldo; Danny M. Cohn; Dorottya Csuka; Henriette Farkas; Mark Gompels; Richard Gower; Anete S. Grumach; Guillermo Guidos-Fogelbach; Michihiro Hide; Hye-Ryun Kang; Allen Phillip Kaplan; Constance Katelaris; Sorena Kiani-Alikhan; Wei-Te Lei; Richard Lockey; Hilary Longhurst; William R. Lumry; Andrew MacGinnitie; Alejandro Malbran; Inmaculada Martinez Saguer; Juan José Matta; 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

doi:10.1111/all.15214

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 Castaldo, Danny M. Cohn, Dorottya CsukaHenriette Farkas, Mark Gompels, Richard Gower, Anete S. Grumach, Guillermo Guidos-Fogelbach, Michihiro Hide, Hye-Ryun Kang, Allen Phillip Kaplan, Constance Katelaris, Sorena Kiani-Alikhan, Wei-Te Lei, Richard Lockey, Hilary Longhurst, William R. Lumry, Andrew MacGinnitie, Alejandro Malbran, Inmaculada Martinez Saguer, Juan José Matta, 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

Western Sydney University

Research output: Contribution to journal › Article › peer-review

308 Citations (Scopus)

Abstract

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.

Original language	English
Pages (from-to)	1961-1990
Number of pages	30
Journal	Allergy: European Journal of Allergy and Clinical Immunology
Volume	77
Issue number	7
DOIs	https://doi.org/10.1111/all.15214
Publication status	Published - Jul 2022

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© 2022 The Authors. Allergy published by European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd.

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This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License (http://creativecommons.org/licenses/by-nc-nd/4.0/), which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

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Maurer, M., Magerl, M., Betschel, S., Aberer, W., Ansotegui, I. J., Aygören-Pürsün, E., Banerji, A., Bara, N.-A., Boccon-Gibod, I., Bork, K., Bouillet, L., Boysen, H. B., Brodszki, N., Busse, P. J., Bygum, A., Caballero, T., Cancian, M., Castaldo, A., Cohn, D. M., ... Craig, T. (2022). The international WAO/EAACI guideline for the management of hereditary angioedema : the 2021 revision and update. Allergy: European Journal of Allergy and Clinical Immunology, 77(7), 1961-1990. https://doi.org/10.1111/all.15214

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title = "The international WAO/EAACI guideline for the management of hereditary angioedema : the 2021 revision and update",

abstract = "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.",

author = "Marcus Maurer and Markus Magerl and Stephen Betschel and Werner Aberer and Ansotegui, {Ignacio J.} and Emel Ayg{\"o}ren-P{\"u}rs{\"u}n and Aleena Banerji and No{\'e}mi-Anna Bara and Isabelle Boccon-Gibod and Konrad Bork and Laurence Bouillet and Boysen, {Henrik Balle} and Nicholas Brodszki and Busse, {Paula J.} and Anette Bygum and Teresa Caballero and Mauro Cancian and Anthony Castaldo and Cohn, {Danny M.} and Dorottya Csuka and Henriette Farkas and Mark Gompels and Richard Gower and Grumach, {Anete S.} and Guillermo Guidos-Fogelbach and Michihiro Hide and Hye-Ryun Kang and Kaplan, {Allen Phillip} and Constance Katelaris and Sorena Kiani-Alikhan and Wei-Te Lei and Richard Lockey and Hilary Longhurst and Lumry, {William R.} and Andrew MacGinnitie and Alejandro Malbran and {Martinez Saguer}, Inmaculada and Matta, {Juan Jos{\'e}} and Alexander Nast and Dinh Nguyen and Nieto-Martinez, {Sandra A.} and Ruby Pawankar and Jonathan Peter and Grzegorz Porebski and Nieves Prior and Avner Reshef and Marc Riedl and Bruce Ritchie and Sheikh, {Farrukh Rafique} and Smith, {William B.} and Spaeth, {Peter J.} and Marcin Stobiecki and Elias Toubi and Varga, {Lilian Agnes} and Karsten Weller and Andrea Zanichelli and Yuxiang Zhi and Bruce Zuraw and Timothy Craig",

note = "Publisher Copyright: {\textcopyright} 2022 The Authors. Allergy published by European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd.",

year = "2022",

month = jul,

doi = "10.1111/all.15214",

language = "English",

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journal = "Allergy: European Journal of Allergy and Clinical Immunology",

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Maurer, M, Magerl, M, Betschel, S, Aberer, W, Ansotegui, IJ, Aygören-Pürsün, E, Banerji, A, Bara, N-A, Boccon-Gibod, I, Bork, K, Bouillet, L, Boysen, HB, Brodszki, N, Busse, PJ, Bygum, A, Caballero, T, Cancian, M, Castaldo, A, Cohn, DM, Csuka, D, Farkas, H, Gompels, M, Gower, R, Grumach, AS, Guidos-Fogelbach, G, Hide, M, Kang, H-R, Kaplan, AP, Katelaris, C, Kiani-Alikhan, S, Lei, W-T, Lockey, R, Longhurst, H, Lumry, WR, MacGinnitie, A, Malbran, A, Martinez Saguer, I, Matta, JJ, Nast, A, Nguyen, D, Nieto-Martinez, SA, Pawankar, R, Peter, J, Porebski, G, Prior, N, Reshef, A, Riedl, M, Ritchie, B, Sheikh, FR, Smith, WB, Spaeth, PJ, Stobiecki, M, Toubi, E, Varga, LA, Weller, K, Zanichelli, A, Zhi, Y, Zuraw, B & Craig, T 2022, 'The international WAO/EAACI guideline for the management of hereditary angioedema : the 2021 revision and update', Allergy: European Journal of Allergy and Clinical Immunology, vol. 77, no. 7, pp. 1961-1990. https://doi.org/10.1111/all.15214

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AU - Maurer, Marcus

AU - Magerl, Markus

AU - Betschel, Stephen

AU - Aberer, Werner

AU - Ansotegui, Ignacio J.

AU - Aygören-Pürsün, Emel

AU - Banerji, Aleena

AU - Bara, Noémi-Anna

AU - Boccon-Gibod, Isabelle

AU - Bork, Konrad

AU - Bouillet, Laurence

AU - Boysen, Henrik Balle

AU - Brodszki, Nicholas

AU - Busse, Paula J.

AU - Bygum, Anette

AU - Caballero, Teresa

AU - Cancian, Mauro

AU - Castaldo, Anthony

AU - Cohn, Danny M.

AU - Csuka, Dorottya

AU - Farkas, Henriette

AU - Gompels, Mark

AU - Gower, Richard

AU - Grumach, Anete S.

AU - Guidos-Fogelbach, Guillermo

AU - Hide, Michihiro

AU - Kang, Hye-Ryun

AU - Kaplan, Allen Phillip

AU - Katelaris, Constance

AU - Kiani-Alikhan, Sorena

AU - Lei, Wei-Te

AU - Lockey, Richard

AU - Longhurst, Hilary

AU - Lumry, William R.

AU - MacGinnitie, Andrew

AU - Malbran, Alejandro

AU - Martinez Saguer, Inmaculada

AU - Matta, Juan José

AU - Nast, Alexander

AU - Nguyen, Dinh

AU - Nieto-Martinez, Sandra A.

AU - Pawankar, Ruby

AU - Peter, Jonathan

AU - Porebski, Grzegorz

AU - Prior, Nieves

AU - Reshef, Avner

AU - Riedl, Marc

AU - Ritchie, Bruce

AU - Sheikh, Farrukh Rafique

AU - Smith, William B.

AU - Spaeth, Peter J.

AU - Stobiecki, Marcin

AU - Toubi, Elias

AU - Varga, Lilian Agnes

AU - Weller, Karsten

AU - Zanichelli, Andrea

AU - Zhi, Yuxiang

AU - Zuraw, Bruce

AU - Craig, Timothy

PY - 2022/7

Y1 - 2022/7

N2 - 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.

AB - 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.

UR - https://hdl.handle.net/1959.7/uws:75434

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DO - 10.1111/all.15214

M3 - Article

SN - 0105-4538

VL - 77

SP - 1961

EP - 1990

JO - Allergy: European Journal of Allergy and Clinical Immunology

JF - Allergy: European Journal of Allergy and Clinical Immunology

IS - 7

ER -

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

Abstract

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