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; Dorrottya 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 R. 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/10.1111/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, Dorrottya 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 R. 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

146 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
Early online date	2022
DOIs	https://doi.org/10.1111/all.15214
Publication status	Published - Jul 2022

Keywords

C1 inhibitor
DELPHI
disease control
guideline
hereditary angioedema
management

Access to Document

https://doi.org/10.1111/all.15214

Cite this

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

@article{b54d23e9931f46459e25ba6406b98bfe,

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.",

keywords = "C1 inhibitor, DELPHI, disease control, guideline, hereditary angioedema, management",

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 Dorrottya 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 {Rafique Sheikh}, Farrukh and Smith, {William R.} 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: 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 GA2LEN/HAEi network of Angioedema Centers of Reference and Excellence (ACARE, https://acare-network.com) The authors would like to thank to Sofia Dorsano and Beate Schinzel for their fantastic support during the process of the guideline development. They would also like to thank the national societies for the support of their delegates. Open access funding enabled and organized by ProjektDEAL. 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, and grants and personal fees from Shire/Takeda, outside the submitted work. Dr. Magerl reports personal fees and nonfinancial support from Shire/Takeda, personal fees and nonfinancial support from CSL Behring, personal fees from Pharming, personal fees and nonfinancial support from Biocryst, during the conduct of the study, personal fees and nonfinancial support from Shire/Takeda, personal fees and nonfinancial support from CSL Behring, personal fees from Pharming, personal fees and nonfinancial support from Biocryst, personal fees from Kalvista, personal fees from Octapharma, personal fees from Novartis, outside the submitted work. Dr Betschel has received fees for advisory boards, presentations and research from CSL and Takeda. Advisor fees from Biocryst and Kalvista, Octapharma, Pharming, outside the submitted work. Dr. Aberer has nothing to disclose. Dr. Ansotegui has nothing to disclose. Dr. Ayg{\"o}ren‐P{\"u}rs{\"u}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. Bara reports personal fees from commercial sponsor, outside the submitted work. Dr. Boccon‐gibod reports grants, personal fees and nonfinancial support from Takeda, grants, personal fees and nonfinancial support from Biocryst Pharmaceutical, nonfinancial 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. H. B. Boysen has nothing to disclose. Dr. Brodski has nothing relevant to disclose. Grants and personal fees from CSL Behring, Biocryst, Takeda; personal fees from Medscape, CVS, Novartis, Regeneraon; grants from Biocryst. Dr. 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. Castaldo has nothing to disclose. 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. Csuka has nothing to disclose. 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. Gompels reports fee for CSL and conference attendance support, member of the immunology clinical reference group, clinical trial work for Novartis, BioCryst. 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 Grumach reports grants from Shire/Takeda and personal fees from CSL Behring, Takeda, and Catalyst for educative programs and consulting. Dr. Guidos‐Fogelbach has nothing to disclose. Dr. Hide reports personal fees from BioCryst, CSL Behring, Takeda, and Torii. Dr. Kang has nothing to disclose. Dr. Kaplan has nothing to disclose. Dr. Katelaris reports grants from CSL Behring, personal fees from CSL Behring, personal fees from Takeda, during the conduct of the study. Dr. Kiani‐Alikhan reports personal fees from BioCryst, personal fees from CSL Behring, personal fees from Takeda, outside the submitted work. Dr. Lei has nothing to disclose. Dr. Lockey has nothing to disclose. 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. MacGinnitie reports personal fees from Biocryst, during the conduct of the study. Dr. Malbran has nothing to disclose. Inmaculada Martinez Saguer has received research grant support and/or speaker/consultancy fees from BioCryst, CSL Behring, KalVista, Pharming, and Takeda. Dr. Matta has nothing to disclose. Dr. Nast has no conflict of interest in relation to this manuscript. Dr. Nguyen has nothing to disclose. Dr. Nieto‐Martinez has nothing to disclose. Dr. Pawankar declares no conflicts of interest in relation to this manuscript. Dr. Peter has nothing to disclose. Dr. Porebski is or recently was a speaker and/or advisor for CSL Behring, Takeda, Pharming and has served as an investigator for clinical trials sponsored by BioCryst Pharmaceuticals. Dr. Prior reports receipt of research grant support and/or speaker/consultancy fees from CSL Behring, Pharming, and Shire (currently Takeda). Dr. Reshef Received research grants, speakers, and consulting honoraria from CSL Behring, Stallergens, Teva, Pharming, BioCryst, Pharvaris, Takeda (Shire)—not related to the article. Dr. Marc Riedl has received research grants from BioCryst, CSL Behring, Ionis, Kalvista, Pharvaris, and Takeda outside the submitted work; consultancy fees from Astria, BioCryst, Biomarin, CSL Behring, Cycle Pharma, Fresenius Kabi, Ipsen, Kalvista, Ono Pharma, Pfizer, Pharming, Pharvaris, RegenexBio, and Takeda outside the submitted work. Dr. Bruce Ritchie has received Research grants from CSL Behring and has participated in clinical trials with Biocrist, CSL Behring, Ionis, Kalvista, Pharvaris, and Takeda. The University of Alberta has received donations in lieu of consultancy fees to Dr. Ritchie from CSL Behring, Takeda. Dr. Sheikh reports other from Takeda, grants from CSL Behring, outside the submitted work. Dr. Smith reports other from BioCryst, during the conduct of the study, personal fees from Takeda/Shire, personal fees from CSL Behring, outside the submitted work. Dr. Sp{\"a}th reports other from CSL Ltd, outside the submitted work, and Expert in an ongoing litigation. Dr. Stobiecki reports other from Takeda, Biocryst, Pharming, Kalvista, fees from CSL Behring, Takeda, Biocryst, outside the submitted work. Dr. Toubi has no conflict of interest in relation to this manuscript. Dr. Varga has no conflict of interest in relation to this manuscript. Dr. Weller reports personal fees from Biocryst, CSL Behring, MOXIE and Pharvaris as well as grants and personal fees from Shire/Takeda, all outside the submitted work. Dr. Zanichelli has no conflict of interest in relation to this manuscript. Dr. Zhi has nothing to disclose. Dr. Zuraw has been a paid consultant for Biomarin, CSL Behring, Cycle Pharma, Fresenius Kabi, and Takeda. He serves on adjudication boards for Novartis and Genentech. Dr. Craig does research for CSL Behring, Ionis, Takeda, Kalvista, Pharvaris, BioMarin, and Biocryst. He speaks for CSL Behring, Takeda, and Grifols. He has a grant to support education from Takeda. He consults with Spark, CSL Behring, Takeda, BioMarin, Pharming, and Biocryst. 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 = "https://doi.org/10.1111/all.15214",

language = "English",

volume = "77",

pages = "1961--1990",

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

issn = "0105-4538",

publisher = "Wiley-Blackwell",

number = "7",

}

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, Rafique Sheikh, F, Smith, WR, 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

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

AU - Cohn, Danny M.

AU - Csuka, Dorrottya

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 - Rafique Sheikh, Farrukh

AU - Smith, William R.

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: 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 GA2LEN/HAEi network of Angioedema Centers of Reference and Excellence (ACARE, https://acare-network.com) The authors would like to thank to Sofia Dorsano and Beate Schinzel for their fantastic support during the process of the guideline development. They would also like to thank the national societies for the support of their delegates. Open access funding enabled and organized by ProjektDEAL. 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, and grants and personal fees from Shire/Takeda, outside the submitted work. Dr. Magerl reports personal fees and nonfinancial support from Shire/Takeda, personal fees and nonfinancial support from CSL Behring, personal fees from Pharming, personal fees and nonfinancial support from Biocryst, during the conduct of the study, personal fees and nonfinancial support from Shire/Takeda, personal fees and nonfinancial support from CSL Behring, personal fees from Pharming, personal fees and nonfinancial support from Biocryst, personal fees from Kalvista, personal fees from Octapharma, personal fees from Novartis, outside the submitted work. Dr Betschel has received fees for advisory boards, presentations and research from CSL and Takeda. Advisor fees from Biocryst and Kalvista, Octapharma, Pharming, outside the submitted work. Dr. Aberer has nothing to disclose. Dr. Ansotegui has nothing to disclose. Dr. Aygören‐Pürsün 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. Bara reports personal fees from commercial sponsor, outside the submitted work. Dr. Boccon‐gibod reports grants, personal fees and nonfinancial support from Takeda, grants, personal fees and nonfinancial support from Biocryst Pharmaceutical, nonfinancial 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. H. B. Boysen has nothing to disclose. Dr. Brodski has nothing relevant to disclose. Grants and personal fees from CSL Behring, Biocryst, Takeda; personal fees from Medscape, CVS, Novartis, Regeneraon; grants from Biocryst. Dr. 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. Castaldo has nothing to disclose. 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. Csuka has nothing to disclose. 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. Gompels reports fee for CSL and conference attendance support, member of the immunology clinical reference group, clinical trial work for Novartis, BioCryst. 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 Grumach reports grants from Shire/Takeda and personal fees from CSL Behring, Takeda, and Catalyst for educative programs and consulting. Dr. Guidos‐Fogelbach has nothing to disclose. Dr. Hide reports personal fees from BioCryst, CSL Behring, Takeda, and Torii. Dr. Kang has nothing to disclose. Dr. Kaplan has nothing to disclose. Dr. Katelaris reports grants from CSL Behring, personal fees from CSL Behring, personal fees from Takeda, during the conduct of the study. Dr. Kiani‐Alikhan reports personal fees from BioCryst, personal fees from CSL Behring, personal fees from Takeda, outside the submitted work. Dr. Lei has nothing to disclose. Dr. Lockey has nothing to disclose. 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. MacGinnitie reports personal fees from Biocryst, during the conduct of the study. Dr. Malbran has nothing to disclose. Inmaculada Martinez Saguer has received research grant support and/or speaker/consultancy fees from BioCryst, CSL Behring, KalVista, Pharming, and Takeda. Dr. Matta has nothing to disclose. Dr. Nast has no conflict of interest in relation to this manuscript. Dr. Nguyen has nothing to disclose. Dr. Nieto‐Martinez has nothing to disclose. Dr. Pawankar declares no conflicts of interest in relation to this manuscript. Dr. Peter has nothing to disclose. Dr. Porebski is or recently was a speaker and/or advisor for CSL Behring, Takeda, Pharming and has served as an investigator for clinical trials sponsored by BioCryst Pharmaceuticals. Dr. Prior reports receipt of research grant support and/or speaker/consultancy fees from CSL Behring, Pharming, and Shire (currently Takeda). Dr. Reshef Received research grants, speakers, and consulting honoraria from CSL Behring, Stallergens, Teva, Pharming, BioCryst, Pharvaris, Takeda (Shire)—not related to the article. Dr. Marc Riedl has received research grants from BioCryst, CSL Behring, Ionis, Kalvista, Pharvaris, and Takeda outside the submitted work; consultancy fees from Astria, BioCryst, Biomarin, CSL Behring, Cycle Pharma, Fresenius Kabi, Ipsen, Kalvista, Ono Pharma, Pfizer, Pharming, Pharvaris, RegenexBio, and Takeda outside the submitted work. Dr. Bruce Ritchie has received Research grants from CSL Behring and has participated in clinical trials with Biocrist, CSL Behring, Ionis, Kalvista, Pharvaris, and Takeda. The University of Alberta has received donations in lieu of consultancy fees to Dr. Ritchie from CSL Behring, Takeda. Dr. Sheikh reports other from Takeda, grants from CSL Behring, outside the submitted work. Dr. Smith reports other from BioCryst, during the conduct of the study, personal fees from Takeda/Shire, personal fees from CSL Behring, outside the submitted work. Dr. Späth reports other from CSL Ltd, outside the submitted work, and Expert in an ongoing litigation. Dr. Stobiecki reports other from Takeda, Biocryst, Pharming, Kalvista, fees from CSL Behring, Takeda, Biocryst, outside the submitted work. Dr. Toubi has no conflict of interest in relation to this manuscript. Dr. Varga has no conflict of interest in relation to this manuscript. Dr. Weller reports personal fees from Biocryst, CSL Behring, MOXIE and Pharvaris as well as grants and personal fees from Shire/Takeda, all outside the submitted work. Dr. Zanichelli has no conflict of interest in relation to this manuscript. Dr. Zhi has nothing to disclose. Dr. Zuraw has been a paid consultant for Biomarin, CSL Behring, Cycle Pharma, Fresenius Kabi, and Takeda. He serves on adjudication boards for Novartis and Genentech. Dr. Craig does research for CSL Behring, Ionis, Takeda, Kalvista, Pharvaris, BioMarin, and Biocryst. He speaks for CSL Behring, Takeda, and Grifols. He has a grant to support education from Takeda. He consults with Spark, CSL Behring, Takeda, BioMarin, Pharming, and Biocryst. Publisher Copyright: © 2022 The Authors. Allergy published by European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd.

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.

KW - C1 inhibitor

KW - DELPHI

KW - disease control

KW - guideline

KW - hereditary angioedema

KW - management

UR - http://www.scopus.com/inward/record.url?scp=85124880250&partnerID=8YFLogxK

U2 - https://doi.org/10.1111/all.15214

DO - https://doi.org/10.1111/all.15214

M3 - Article

C2 - 35006617

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

Keywords

Access to Document

Other files and links

Cite this