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 Csuka; Henriette 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

doi:https://doi.org/10.1016/j.waojou.2022.100627

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 journal › Article › Academic › peer-review

51 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? 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
Article number	100627
Journal	World Allergy Organization Journal
Volume	15
Issue number	3
DOIs	https://doi.org/10.1016/j.waojou.2022.100627
Publication status	Published - 1 Mar 2022

Keywords

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

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https://doi.org/10.1016/j.waojou.2022.100627

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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. J., Cohn, D. M., ... Craig, T. (2022). The international WAO/EAACI guideline for the management of hereditary angioedema – The 2021 revision and update. World Allergy Organization Journal, 15(3), Article 100627. https://doi.org/10.1016/j.waojou.2022.100627

@article{1059272bfaaf49adad857dce2db93042,

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? 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.",

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

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 Castaldo, {Anthony J.} 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 P.} and Katelaris, {Constance H.} and Sorena Kiani-Alikhan and Wei-Te Lei and Lockey, {Richard F.} and Hilary Longhurst and William Lumry and Andrew MacGinnitie and Alejandro Malbran and {Martinez Saguer}, Inmaculada and {Matta Campos}, {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 = "Funding Information: Dr. Maurer reports personal fees from Alnylam , grants and personal fees from BioCryst , grants from Centogene , grants and personal fees from CSL Behring , grants from Dyax , grants and personal fees from Kalvista , grants from Pharming , personal fees from Pharvaris , grants and personal fees from Shire/Takeda , outside the submitted work. Funding Information: Dr. Maurer reports personal fees from Alnylam, grants and personal fees from BioCryst, grants from Centogene, grants and personal fees from CSL Behring, grants from Dyax, grants and personal fees from Kalvista, grants from Pharming, personal fees from Pharvaris, grants and personal fees from Shire/Takeda, outside the submitted work.Dr. Magerl reports personal fees and non-financial support from Shire/Takeda, personal fees and non-financial support from CSL Behring, personal fees from Pharming, personal fees and non-financial support from Biocryst, during the conduct of the study; personal fees and non-financial support from Shire/Takeda, personal fees and non-financial support from CSL Behring, personal fees from Pharming, personal fees and non-financial support from Biocryst, personal fees from Kalvista, personal fees from Octapharma, personal fees from Novartis, outside the submitted work.Dr. Ayg?ren-P?rs?n reports personal fees from Biocryst, personal fees from Biomarin, personal fees from Centogene, grants and personal fees from CSL Behring, personal fees from Kalvista, personal fees from Pharming, personal fees from Pharvaris, grants and personal fees from Shire/Takeda, outside the submitted work.Dr. Banerji reports grants from Takeda, Biocryst, other from Takeda, Biocryst, Pharming, CSL, Kalvista, Pharvaris, outside the submitted work.Dr. Boccon-gibod reports grants, personal fees and non-financial support from Takeda, grants, personal fees and non-financial support from Biocryst Pharmaceutical, non-financial support from Pharming, outside the submitted work.Dr. Bork reports grants and personal fees from CSL Behring, personal fees from Shire/Takeda, personal fees from Pharvaris, outside the submitted work.Dr. Bouillet reports grants and personal fees from Takeda, Biocryst, Novartis, and GSK.Dr. Busse reports grants and personal fees from CSL Behring, BioCryst, Takeda; personal fees from Medscape, CVS Health, Novartis, RegeneronDr. Bygum reports grants and personal fees from CSL Behring, grants from BioCryst, personal fees from Takeda/Shire outside the submitted work.Dr. Caballero reports grants, personal fees and other from CSL-BEHRING, grants, personal fees and other from TAKEDA, personal fees and other from PHARMING NV, personal fees from OCTAPHARMA, personal fees and other from BIOCRYST, personal fees and other from NOVARTIS, outside the submitted work.Dr. Cancian reports and has received grant research support and/or speaker/consultancy fees from BioCryst, CSL Behring, Kalvista, Pharming, Shire (a Takeda company) and SOBI; has received funding to attend conferences/educational events from CSL Behring, Menarini, MSD, Novartis, Pharming and Shire; is/has been a clinical trial/registry investigator for BioCryst, CSL Behring, Kalvista, Novartis, Pharming, Shire and UCB.Dr. Cohn reports personal fees from CSL Behring, personal fees from Shire/Takeda, personal fees from Ionis Pharmaceuticals, Inc, personal fees from KalVista, personal fees from BioCryst, personal fees from Pharming, personal fees from Pharvaris, personal fees from Sanofi/Genzyme Europe, outside the submitted work.Dr. Farkas reports grants and personal fees from CSL Behring, grants and personal fees from Shire/Takeda, grants and personal fees from Pharming Group NV, personal fees from Biocryst Pharmaceuticals, personal fees from Kalvista, personal fees from ONO Pharmaceuticals, outside the submitted work.Dr. Gower reports Industry funded research/investigator: BioCryst, Kalvista, Pharming, Pharvaris, Takeda/Shire/Dyax Consultant: BioCryst, CSL Behring, Fresenius Kabi, Pharming, Takeda/Shire/Dyax Speakers Bureau, Faculty, Peer Reviewer: BioCryst, Pharming, Takeda/Shire/Dyax Advisory Committee/Board: BioCryst, CSL Behring, Fresenius Kabi, Pharming, Takeda/Shire/Dyax.Dr Hide reports personal fees from BioCryst, CSL Behring, Takeda and Torii.Dr. Longhurst has consulted for, acted as speaker for or collaborated in research with the following: Adverum, BioCryst, CSL Behring, GSK, Intellia, Ionis, Kalivista, Pharming, Pharvaris, and Takeda.Dr. Lumry has consulted for, acted as speaker for or collaborated in research with the following: Adverum, Astra Zeneca, BioCryst, Biomarin, CSL Behring, Fresenius Kabi, GSK, Intellia, Ionis, Kalivista, Pharming, Pharvaris, Sanofi Regeneron, Takeda and Teva.Dr. Reshef Received research grants, speakers and consulting honoraria from CSL Behring, Stallergens, Teva, Pharming, BioCryst, Pharvaris, Takeda (Shire) - not related to the article. Funding Information: Dr. Bygum reports grants and personal fees from CSL Behring , grants from BioCryst , personal fees from Takeda/Shire outside the submitted work. Funding Information: Dr. Cancian reports and has received grant research support and/or speaker/consultancy fees from BioCryst , CSL Behring , Kalvista , Pharming , Shire (a Takeda company) and SOBI ; has received funding to attend conferences/educational events from CSL Behring, Menarini , MSD , Novartis , Pharming and Shire ; is/has been a clinical trial/registry investigator for BioCryst, CSL Behring, Kalvista, Novartis, Pharming, Shire and UCB. Funding Information: Dr Grumach reports grants from Shire/Takeda and personal fees from CSL Behring, Takeda, and Catalyst for educative programs and consulting. Publisher Copyright: {\textcopyright} 2022 The Author(s)",

year = "2022",

month = mar,

day = "1",

doi = "https://doi.org/10.1016/j.waojou.2022.100627",

language = "English",

volume = "15",

journal = "World Allergy Organization Journal",

issn = "1939-4551",

publisher = "BioMed Central Ltd.",

number = "3",

}

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, AJ, Cohn, DM, Csuka, D, Farkas, H, Gompels, M, Gower, R, Grumach, AS, Guidos-Fogelbach, G, Hide, M, Kang, H-R, Kaplan, AP, Katelaris, CH, Kiani-Alikhan, S, Lei, W-T, Lockey, RF, Longhurst, H, Lumry, W, MacGinnitie, A, Malbran, A, Martinez Saguer, I, Matta Campos, 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', World Allergy Organization Journal, vol. 15, no. 3, 100627. https://doi.org/10.1016/j.waojou.2022.100627

TY - JOUR

T1 - The international WAO/EAACI guideline for the management of hereditary angioedema – The 2021 revision and update

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 J.

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 P.

AU - Katelaris, Constance H.

AU - Kiani-Alikhan, Sorena

AU - Lei, Wei-Te

AU - Lockey, Richard F.

AU - Longhurst, Hilary

AU - Lumry, William

AU - MacGinnitie, Andrew

AU - Malbran, Alejandro

AU - Martinez Saguer, Inmaculada

AU - Matta Campos, 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

N1 - Funding Information: Dr. Maurer reports personal fees from Alnylam , grants and personal fees from BioCryst , grants from Centogene , grants and personal fees from CSL Behring , grants from Dyax , grants and personal fees from Kalvista , grants from Pharming , personal fees from Pharvaris , grants and personal fees from Shire/Takeda , outside the submitted work. Funding Information: Dr. Maurer reports personal fees from Alnylam, grants and personal fees from BioCryst, grants from Centogene, grants and personal fees from CSL Behring, grants from Dyax, grants and personal fees from Kalvista, grants from Pharming, personal fees from Pharvaris, grants and personal fees from Shire/Takeda, outside the submitted work.Dr. Magerl reports personal fees and non-financial support from Shire/Takeda, personal fees and non-financial support from CSL Behring, personal fees from Pharming, personal fees and non-financial support from Biocryst, during the conduct of the study; personal fees and non-financial support from Shire/Takeda, personal fees and non-financial support from CSL Behring, personal fees from Pharming, personal fees and non-financial support from Biocryst, personal fees from Kalvista, personal fees from Octapharma, personal fees from Novartis, outside the submitted work.Dr. Ayg?ren-P?rs?n reports personal fees from Biocryst, personal fees from Biomarin, personal fees from Centogene, grants and personal fees from CSL Behring, personal fees from Kalvista, personal fees from Pharming, personal fees from Pharvaris, grants and personal fees from Shire/Takeda, outside the submitted work.Dr. Banerji reports grants from Takeda, Biocryst, other from Takeda, Biocryst, Pharming, CSL, Kalvista, Pharvaris, outside the submitted work.Dr. Boccon-gibod reports grants, personal fees and non-financial support from Takeda, grants, personal fees and non-financial support from Biocryst Pharmaceutical, non-financial support from Pharming, outside the submitted work.Dr. Bork reports grants and personal fees from CSL Behring, personal fees from Shire/Takeda, personal fees from Pharvaris, outside the submitted work.Dr. Bouillet reports grants and personal fees from Takeda, Biocryst, Novartis, and GSK.Dr. Busse reports grants and personal fees from CSL Behring, BioCryst, Takeda; personal fees from Medscape, CVS Health, Novartis, RegeneronDr. Bygum reports grants and personal fees from CSL Behring, grants from BioCryst, personal fees from Takeda/Shire outside the submitted work.Dr. Caballero reports grants, personal fees and other from CSL-BEHRING, grants, personal fees and other from TAKEDA, personal fees and other from PHARMING NV, personal fees from OCTAPHARMA, personal fees and other from BIOCRYST, personal fees and other from NOVARTIS, outside the submitted work.Dr. Cancian reports and has received grant research support and/or speaker/consultancy fees from BioCryst, CSL Behring, Kalvista, Pharming, Shire (a Takeda company) and SOBI; has received funding to attend conferences/educational events from CSL Behring, Menarini, MSD, Novartis, Pharming and Shire; is/has been a clinical trial/registry investigator for BioCryst, CSL Behring, Kalvista, Novartis, Pharming, Shire and UCB.Dr. Cohn reports personal fees from CSL Behring, personal fees from Shire/Takeda, personal fees from Ionis Pharmaceuticals, Inc, personal fees from KalVista, personal fees from BioCryst, personal fees from Pharming, personal fees from Pharvaris, personal fees from Sanofi/Genzyme Europe, outside the submitted work.Dr. Farkas reports grants and personal fees from CSL Behring, grants and personal fees from Shire/Takeda, grants and personal fees from Pharming Group NV, personal fees from Biocryst Pharmaceuticals, personal fees from Kalvista, personal fees from ONO Pharmaceuticals, outside the submitted work.Dr. Gower reports Industry funded research/investigator: BioCryst, Kalvista, Pharming, Pharvaris, Takeda/Shire/Dyax Consultant: BioCryst, CSL Behring, Fresenius Kabi, Pharming, Takeda/Shire/Dyax Speakers Bureau, Faculty, Peer Reviewer: BioCryst, Pharming, Takeda/Shire/Dyax Advisory Committee/Board: BioCryst, CSL Behring, Fresenius Kabi, Pharming, Takeda/Shire/Dyax.Dr Hide reports personal fees from BioCryst, CSL Behring, Takeda and Torii.Dr. Longhurst has consulted for, acted as speaker for or collaborated in research with the following: Adverum, BioCryst, CSL Behring, GSK, Intellia, Ionis, Kalivista, Pharming, Pharvaris, and Takeda.Dr. Lumry has consulted for, acted as speaker for or collaborated in research with the following: Adverum, Astra Zeneca, BioCryst, Biomarin, CSL Behring, Fresenius Kabi, GSK, Intellia, Ionis, Kalivista, Pharming, Pharvaris, Sanofi Regeneron, Takeda and Teva.Dr. Reshef Received research grants, speakers and consulting honoraria from CSL Behring, Stallergens, Teva, Pharming, BioCryst, Pharvaris, Takeda (Shire) - not related to the article. Funding Information: Dr. Bygum reports grants and personal fees from CSL Behring , grants from BioCryst , personal fees from Takeda/Shire outside the submitted work. Funding Information: Dr. Cancian reports and has received grant research support and/or speaker/consultancy fees from BioCryst , CSL Behring , Kalvista , Pharming , Shire (a Takeda company) and SOBI ; has received funding to attend conferences/educational events from CSL Behring, Menarini , MSD , Novartis , Pharming and Shire ; is/has been a clinical trial/registry investigator for BioCryst, CSL Behring, Kalvista, Novartis, Pharming, Shire and UCB. Funding Information: Dr Grumach reports grants from Shire/Takeda and personal fees from CSL Behring, Takeda, and Catalyst for educative programs and consulting. Publisher Copyright: © 2022 The Author(s)

PY - 2022/3/1

Y1 - 2022/3/1

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? 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? 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.

KW - C1-inhibitor

KW - DELPHI

KW - GRADE therapy

KW - Hereditary angioedema

KW - diagnosis

KW - disease control

KW - guideline

KW - management

KW - prophylaxis

KW - quality of life

KW - recommendations

KW - self-administration

UR - http://www.scopus.com/inward/record.url?scp=85127772980&partnerID=8YFLogxK

U2 - https://doi.org/10.1016/j.waojou.2022.100627

DO - https://doi.org/10.1016/j.waojou.2022.100627

M3 - Article

C2 - 35497649

SN - 1939-4551

VL - 15

JO - World Allergy Organization Journal

JF - World Allergy Organization Journal

IS - 3

M1 - 100627

ER -

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

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Keywords

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