Abstract
Hereditary Angioedema (HAE) is a rare and disabling disease. Early diagnosis and appropriate therapy are essential. This update and revision of the global guideline for HAE provides up-to-date consensus recommendations for the management of HAE. In the development of this update and revision of the guideline, an international expert panel reviewed the existing evidence and developed 20 recommendations that were discussed, finalized and consented during the guideline consensus conference in June 2016 in Vienna. The final version of this update and revision of the guideline incorporates the contributions of a board of expert reviewers and the endorsing societies. The goal of this guideline update and revision is to provide clinicians and their patients with guidance that will assist them in making rational decisions in the management of HAE with deficient C1-inhibitor (type 1) and HAE with dysfunctional C1-inhibitor (type 2). The key clinical questions covered by these recommendations are: 1) How should HAE-1/2 be defined and classified?, 2) How should HAE-1/2 be diagnosed?, 3) Should HAE-1/2 patients receive prophylactic and/or on-demand treatment and what treatment options should be used?, 4) Should HAE-1/2 management be different for special HAE-1/2 patient groups such as pregnant/lactating women or children?, and 5) Should HAE-1/2 management incorporate self-administration of therapies and patient support measures? This article is co-published with permission in Allergy and the World Allergy Organization Journal.
Original language | English (US) |
---|---|
Article number | 5 |
Journal | World Allergy Organization Journal |
Volume | 11 |
Issue number | 1 |
DOIs | |
State | Published - Feb 27 2018 |
All Science Journal Classification (ASJC) codes
- Immunology and Allergy
- Immunology
- Pulmonary and Respiratory Medicine
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In: World Allergy Organization Journal, Vol. 11, No. 1, 5, 27.02.2018.
Research output: Contribution to journal › Article › peer-review
TY - JOUR
T1 - The international WAO/EAACI guideline for the management of hereditary angioedema - The 2017 revision and update
AU - Maurer, Marcus
AU - Magerl, Markus
AU - Ansotegui, Ignacio
AU - Aygören-Pürsün, Emel
AU - Betschel, Stephen
AU - Bork, Konrad
AU - Bowen, Tom
AU - Boysen, Henrik Balle
AU - Farkas, Henriette
AU - Grumach, Anete S.
AU - Hide, Michihiro
AU - Katelaris, Constance
AU - Lockey, Richard
AU - Longhurst, Hilary
AU - Lumry, William R.
AU - Martinez-Saguer, Inmaculada
AU - Moldovan, Dumitru
AU - Nast, Alexander
AU - Pawankar, Ruby
AU - Potter, Paul
AU - Riedl, Marc
AU - Ritchie, Bruce
AU - Rosenwasser, Lanny
AU - Sánchez-Borges, Mario
AU - Zhi, Yuxiang
AU - Zuraw, Bruce
AU - Craig, Timothy
N1 - Funding Information: I. Ansotegui has no conflict of interest. E. Aygören-Pürsün has received honoraria as a speaker / advisor and/or is a recipient of institutional research/study funding from BioCryst, CSL Behring, Jerini, Shire, Pharming Technologies and/or Viropharma. SD Betschel is or has been a speaker/consultant and has received research funding currently or in the past from CSL Behring, Shire and Viropharma. K. Bork is a speaker for CSL Behring and Shire. T. Bowen has no conflict of interest. H. B. Boysen is the executive director of HAEi, who has worked and is working with the majority of pharmaceutical companies in the field of hereditary angioedema (HAE). T Craig is a speaker for CSL Behrings, Grifols and Shire. He performs research for Biocryst, CSL Behring, Grifols and Shire. He is a consultant for Biocryst and CSL Behring. He has received educational grant from Shire. H. Farkas received honoraria speaker fee and travel grants from CSL Behring, Shire, Swedish Orphan Biovitrum and Pharming and/or served as a consultant for these companies. A S Grumach is or has been a speaker/consultant for CSL Behring, Shire and Biocryst and performs research with a grant from the Shire Research Program for Investigators. M. Hide has received honoraria as a speaker/adivisor from BioCryst, CSL-Behrin, Shire, and Biropharma. He has received institutional research funding from CSL Behring. Allen Kaplan Consultant for adjudication of angioedema episodes occurring during clinical studies for Genentech. Speaker program on HAE for Shire. CH Katelaris has received honoraria as a speaker and advisory board chair for Shire and CSLBehring and is a principal investigator for trials conducted by CSL Behring, Shire, Biocryst. R. F. Lockey is an investigator for Shire, no other conflict of interest. H.J. Longhurst has received honoraria as a speaker / advisor and/or is a recipient of institutional research/study funding from BioCryst, CSL Behring, Jerini, Shire, and/or Viropharma. WR Lumry is or has been a speaker/consultant and has received research grants from BioCryst, CSL Behring, Dyax, Jerini, Pharming, Shire and Viropharma. M. Magerl is or has been a speaker/consultant for BioCryst, CSL Behring, Jerini, Shire, Sobi, Viropharma. I. Martinez Saguer is or has been a speaker/consultant and performs research for CSL Behring, Shire, Biocryst, Sobi, Viropharma. M. Maurer has received honoraria as a speaker / advisor and/or is a recipient of institutional research/study funding from BioCryst, CSL Behring, Jerini, Shire, and/or Viropharma. Funding Information: aDepartment of Dermatology, Medical University of Graz, Graz, Austria bAngioedema Center, Sheba Medical Center, Israel cDivision of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Boston, Massachusetts, USA dCentre national de référence des angioedèmes (CREAK), Pôle Pluridisciplinaire de Médecine et de Gérontologie Clinique, Grenoble, France eInternal medicine department, national reference center for angioedema, Grenoble University Hospital, Grenoble Alpes (CHUGA), University (UGA), France fDivision of Clinical Immunology, Icahn School at Mount Sinai, New York, USA gAllergy Department, Hospital La Paz Health Research Center (IdiPaz), CIBERER (U754), Madrid, Spain hHungarian Angioedema Center, 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary iDepartment of Immunology, North Bristol NHS Trust, Southmead Hospital, Bristol, UK jMarycliff Clinical Research, Spokane Washington, USA kNational School of Medicine, Instituto Politécnico Nacional, Mexico City, Mexico lDepartment of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore mMedical University of South Carolina, Charleston, SC, USA nDivision of Immunology, Boston Children’s Hospital, Department of Pediatrics, Harvard Medical School, Boston, USA oUnidad de Alergia, Asma e Inmunología Clínica, Buenos Aires pH. Especialidades C.M.N.SXXI, I.M.S.S., México City, México qGenetic Nutrition Unit. Instituto Nacional de Pediatria. Mexico City. Mexico rAllergy Department Hospital Universitario Severo Ochoa, Leganés, Madrid (Spain) sDepartment of clinical Immunology and Allergy, Royal Adelaide Hospital, South Australia tInstitute of Pharmacology, University of Bern, CH-3010 Bern, Switzerland uDepartment of Pediatrics, Division of Pediatric Allergy, Immunology Rheumatology, Hsinchu Mackay Memorial Hospital, Taiwan vDivision of Allergy and Clinical Immunology, Bnai-Zion Medical Center-Haifa Israel wHungarian Angioedema Center, 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary xDepartment of Biomedical and Clinical Sciences, Luigi Sacco, University of Milan – ASST Fatebenefratelli Sacco, Milan, Italy Funding Information: American Academy of Allergy Asthma and Immunology, American College of Allergy Asthma and Immunology, Argentine Association ofAllergy and Clinical Immunology, Asia Pacific Association for Allergy Asthma and Clinical Immunology, Australasian Society of Clinical Immunology and Allergy, Austrian Society of Allergology and Immunology, Azerbaijan Society for Asthma, Allergy and Clinical Immunology, Belarus Association of Allergology & Clinical Immunology, Belgian Society of Allergy and Clinical Immunology, Brazilian Society of Allergy and Immunology, Canadian Society of Allergy and Immunology, Chinese College of Allergy and Asthma, Chinese Society of Allergology, Croatian Society of Allergology and Clinical Immunology, Czech Society of Allergology and Clinical Immunology, Egyptian Society of Allergy and Clinical Immunology, Egyptian Society of Pediatric Allergy and Immunology, German Society for Angioedema, German Society for Dermatology, German Society of Internal Medicine, German Society for Pediatrics & Adolescent Medicine (DGKJ), HAEi (International Organization for C1 Inhibitor Deficiencies), HAEUK, Hungarian Allergy and Immunology Society, Indian Academy of Allergy, Italian Society for Allergology and Clinical Immunology, Japanese Dermatological Association, Japanese Society of Allergology, Korean Academy of Allergy, Asthma and Clinical Immunology, Kuwait Society of Allergy and Clinical Immunology, Malaysian Society of Allergy and Immunology, Polish Society of Allergology, Portuguese Society of Allergology and Clinical Immunology, Romanian Network for Hereditary Angioedema (RHAEN), Spanish Society of Allergology and Clinical Immunology, Ukrainian Allergists Association, Venezuelan Society of Allergy, Asthma, and Immunology. Funding Information: D. Moldovan has ties with CSL Behring, BioCryst, Pharming Technologies, Shire HGT and Swedish Orphan Biovitrum. A. Nast has no conflict of interest. R. Pawankar has no conflict of interest. M. Riedl has received honoraria as a speaker / advisor for Adverum, Alnylam, Arrowhead, BioCryst, CSL Behring, Kalvista, Ionis, Shire, and/or Pharming. He is a recipient of institutional research/study funding from BioCryst, CSL Behring, Ionis, Pharming, and Shire. M. Sánchez-Borges has no conflict of interest. Yuxiang Zhi has no disclosure of potential conflicts of interest. Publisher Copyright: © 2018 The Author(s).
PY - 2018/2/27
Y1 - 2018/2/27
N2 - Hereditary Angioedema (HAE) is a rare and disabling disease. Early diagnosis and appropriate therapy are essential. This update and revision of the global guideline for HAE provides up-to-date consensus recommendations for the management of HAE. In the development of this update and revision of the guideline, an international expert panel reviewed the existing evidence and developed 20 recommendations that were discussed, finalized and consented during the guideline consensus conference in June 2016 in Vienna. The final version of this update and revision of the guideline incorporates the contributions of a board of expert reviewers and the endorsing societies. The goal of this guideline update and revision is to provide clinicians and their patients with guidance that will assist them in making rational decisions in the management of HAE with deficient C1-inhibitor (type 1) and HAE with dysfunctional C1-inhibitor (type 2). The key clinical questions covered by these recommendations are: 1) How should HAE-1/2 be defined and classified?, 2) How should HAE-1/2 be diagnosed?, 3) Should HAE-1/2 patients receive prophylactic and/or on-demand treatment and what treatment options should be used?, 4) Should HAE-1/2 management be different for special HAE-1/2 patient groups such as pregnant/lactating women or children?, and 5) Should HAE-1/2 management incorporate self-administration of therapies and patient support measures? This article is co-published with permission in Allergy and the World Allergy Organization Journal.
AB - Hereditary Angioedema (HAE) is a rare and disabling disease. Early diagnosis and appropriate therapy are essential. This update and revision of the global guideline for HAE provides up-to-date consensus recommendations for the management of HAE. In the development of this update and revision of the guideline, an international expert panel reviewed the existing evidence and developed 20 recommendations that were discussed, finalized and consented during the guideline consensus conference in June 2016 in Vienna. The final version of this update and revision of the guideline incorporates the contributions of a board of expert reviewers and the endorsing societies. The goal of this guideline update and revision is to provide clinicians and their patients with guidance that will assist them in making rational decisions in the management of HAE with deficient C1-inhibitor (type 1) and HAE with dysfunctional C1-inhibitor (type 2). The key clinical questions covered by these recommendations are: 1) How should HAE-1/2 be defined and classified?, 2) How should HAE-1/2 be diagnosed?, 3) Should HAE-1/2 patients receive prophylactic and/or on-demand treatment and what treatment options should be used?, 4) Should HAE-1/2 management be different for special HAE-1/2 patient groups such as pregnant/lactating women or children?, and 5) Should HAE-1/2 management incorporate self-administration of therapies and patient support measures? This article is co-published with permission in Allergy and the World Allergy Organization Journal.
UR - http://www.scopus.com/inward/record.url?scp=85042715589&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85042715589&partnerID=8YFLogxK
U2 - 10.1186/s40413-017-0180-1
DO - 10.1186/s40413-017-0180-1
M3 - Article
AN - SCOPUS:85042715589
SN - 1939-4551
VL - 11
JO - World Allergy Organization Journal
JF - World Allergy Organization Journal
IS - 1
M1 - 5
ER -