World Allergy Organization Journal (Mar 2022)
The international WAO/EAACI guideline for the management of hereditary angioedema – The 2021 revision and update
- Marcus Maurer, MD,
- Markus Magerl, MD,
- Stephen Betschel, MD,
- Werner Aberer, MD,
- Ignacio J. Ansotegui, MD, PhD,
- Emel Aygören-Pürsün, MD,
- Aleena Banerji, MD,
- Noémi-Anna Bara, MD,
- Isabelle Boccon-Gibod, MD,
- Konrad Bork, MD,
- Laurence Bouillet, Pr, MD, PhD,
- Henrik Balle Boysen,
- Nicholas Brodszki, MD, PhD,
- Paula J. Busse, MD,
- Anette Bygum, MD, DMSci,
- Teresa Caballero, MD, PhD,
- Mauro Cancian, MD, PhD,
- Anthony J. Castaldo,
- Danny M. Cohn, MD, PhD,
- Dorottya Csuka, MD,
- Henriette Farkas, MD, PhD, DSc,
- Mark Gompels, MBBS, BSc, MD,
- Richard Gower, MD,
- Anete S. Grumach, MD, PhD,
- Guillermo Guidos-Fogelbach, MD, PhD,
- Michihiro Hide, MD, PhD,
- Hye-Ryun Kang, MD, PhD,
- Allen P. Kaplan, MD,
- Constance H. Katelaris, MBBS, PhD,
- Sorena Kiani-Alikhan, PhD,
- Wei-Te Lei, MD,
- Richard F. Lockey, MD,
- Hilary Longhurst, PhD,
- William Lumry, MD,
- Andrew MacGinnitie, MD, PhD,
- Alejandro Malbran, MD, PhD,
- Inmaculada Martinez Saguer, MD,
- Juan José Matta Campos, MD,
- Alexander Nast, MD,
- Dinh Nguyen, MD, PhD,
- Sandra A. Nieto-Martinez, MD,
- Ruby Pawankar, MD, PhD,
- Jonathan Peter, MB ChB, MMed, FCP (SA), PhD,
- Grzegorz Porebski, MD,
- Nieves Prior, MD, PhD,
- Avner Reshef, MD,
- Marc Riedl, MD,
- Bruce Ritchie, MD,
- Farrukh Rafique Sheikh, MBBS,
- William B. Smith, MBBS, PhD,
- Peter J. Spaeth, PhD,
- Marcin Stobiecki, MD,
- Elias Toubi, MD,
- Lilian Agnes Varga, PhD,
- Karsten Weller, MD,
- Andrea Zanichelli, MD,
- Yuxiang Zhi, MD,
- Bruce Zuraw, MD,
- Timothy Craig, MD
Affiliations
- Marcus Maurer, MD
- Institute of Allergology, Charité–Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.; Frauhofer Institute for Translational Medicine and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany; Corresponding and
- Markus Magerl, MD
- Institute of Allergology, Charité–Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.; Frauhofer Institute for Translational Medicine and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany; co-corresponding authors. Marcus Maurer and Markus Magerl. Institute of Allergology, Charité–Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany. Frauhofer Institute for Translational Medicine and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany.
- Stephen Betschel, MD
- University of Toronto, Toronto, Ontario, Canada
- Werner Aberer, MD
- Department of Dermatology, Medical University of Graz, Graz, Austria
- Ignacio J. Ansotegui, MD, PhD
- Department of Allergy & Immunology, Hospital Quironsalúd Bizkaia, Bilbao-Errandio, Spain
- Emel Aygören-Pürsün, MD
- Center for Children and Adolescents, University Hospital Frankfurt, Frankfurt, Germany
- Aleena Banerji, MD
- Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Boston, MA, United States
- Noémi-Anna Bara, MD
- Romanian Hereditary Angioedema Expertise Centre, Mediquest Clinical Research Center, Sangeorgiu de Mures, Romania
- Isabelle Boccon-Gibod, MD
- National Reference Center for Angioedema (CREAK), Angioedema Center of Reference and Excellence (ACARE), Grenoble Alpes, France; University Hospital, Grenoble, France
- Konrad Bork, MD
- Department of Dermatology, University Medical Center, Johannes Gutenberg University, Mainz, Germany
- Laurence Bouillet, Pr, MD, PhD
- National Reference Center for Angioedema (CREAK), Angioedema Center of Reference and Excellence (ACARE), Grenoble Alpes, France; University Hospital, Grenoble, France
- Henrik Balle Boysen
- HAE International (HAEi), Skanderborg, Denmark
- Nicholas Brodszki, MD, PhD
- Department of Pediatric Immunology, Childrens Hospital, Skåne University Hospital, Lund, Sweden
- Paula J. Busse, MD
- Icahn School of Medicine at Mount Sinai, New York, NY, United States
- Anette Bygum, MD, DMSci
- Clinical Institute, University of Southern Denmark, Odense, Denmark; Department of Clinical Genetics, Odense University Hospital, Odense, Denmark
- Teresa Caballero, MD, PhD
- Allergy Department, Hospital Universitario La Paz, IdiPaz, CIBERER U754, Madrid, Spain
- Mauro Cancian, MD, PhD
- Department of Systems Medicine, University Hospital of Padua, Padua, Italy
- Anthony J. Castaldo
- HAE International (HAEi), Skanderborg, Denmark
- Danny M. Cohn, MD, PhD
- Department of Vascular Medicine, Amsterdam UMC/University of Amsterdam, Amsterdam, the Netherlands
- Dorottya Csuka, MD
- Department of Internal Medicine and Haematology, Hungarian Angioedema Center of Reference and Excellence, Semmelweis University, Budapest, Hungary
- Henriette Farkas, MD, PhD, DSc
- Department of Internal Medicine and Haematology, Hungarian Angioedema Center of Reference and Excellence, Semmelweis University, Budapest, Hungary
- Mark Gompels, MBBS, BSc, MD
- Clinical Immunology, North Bristol NHS Trust, Bristol, United Kingdom
- Richard Gower, MD
- Marycliff Clinical Research, Principle Research Solutions, Spokane, WA, United States
- Anete S. Grumach, MD, PhD
- Clinical Immunology, Centro Universitario FMABC, Sao Paulo, Brazil
- Guillermo Guidos-Fogelbach, MD, PhD
- Instituto Politécnico Nacional SEPI-ENMH, Mexico City, Mexico
- Michihiro Hide, MD, PhD
- Department of Dermatology, Hiroshima Citizens Hospital, Hiroshima, Japan; Department of Dermatology, Hiroshima University, Hiroshima, Japan
- Hye-Ryun Kang, MD, PhD
- Department of Internal Medicine, Seoul National University College of Medicine, Seoul, South Korea
- Allen P. Kaplan, MD
- Division of Pulmonary, Critical Care, Allergy and Immunology, Medical University of South Carolina, Charleston, SC, United States
- Constance H. Katelaris, MBBS, PhD
- Department of Medicine, Campbelltown Hospital and Western Sydney University, Sydney, NSW, Australia
- Sorena Kiani-Alikhan, PhD
- Department of Immunology, Barts Health NHS Trust, London, United Kingdom
- Wei-Te Lei, MD
- Division of Allergy, Immunology, and Rheumatology, Department of Pediatrics, Mackay Memorial Hospital, Hsinchu, Taiwan
- Richard F. Lockey, MD
- Division of Allergy and Immunology, Department of Internal Medicine, Morsani College of Medicine, University of South Florida, Tampa, FL, United States
- Hilary Longhurst, PhD
- Department of Immunology, Auckland District Health Board and Department of Medicine, University of Auckland, Auckland, New Zealand
- William Lumry, MD
- Internal Medicine, Allergy Division, University of Texas Health Science Center, Dallas, TX, United States
- Andrew MacGinnitie, MD, PhD
- Division of Immunology, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States
- Alejandro Malbran, MD, PhD
- Unidad de Alergia, Asma e Inmunología Clínica, Buenos Aires, Argentina
- Inmaculada Martinez Saguer, MD
- Pediatrics, Haemophilia Centre Rhine Main (HZRM), Moerfelden-Walldorf, Germany
- Juan José Matta Campos, MD
- UMAE Hospital Especialidades C.M.N.SXXI, I.M.S.S., México City, Mexico
- Alexander Nast, MD
- Department of Dermatology, Venereology and Allergology, Division of Evidence-Based Medicine Charité–Universitätsmedizin, Berlin, Germany; Corporate Member of Free University of Berlin, Humboldt University of Berlin, Berlin Institute of Health, Berlin, Germany
- Dinh Nguyen, MD, PhD
- Respiratory, Allergy and Clinical Immunology Unit, Internal Medicine Department, Vinmec Healthcare System, College of Health Sciences, VinUniversity, Hanoi, Viet Nam
- Sandra A. Nieto-Martinez, MD
- Genetic Unit of Nutrition, National Institute of Pediatrics, Mexico City, Mexico
- Ruby Pawankar, MD, PhD
- Department of Pediatrics, Nippon Medical School, Tokyo, Japan
- Jonathan Peter, MB ChB, MMed, FCP (SA), PhD
- Division of Allergy and Clinical Immunology, University of Cape Town, Cape Town, South Africa; Allergy and Immunology Unit, University of Cape Town Lung Institute, Cape Town, South Africa
- Grzegorz Porebski, MD
- Department of Clinical and Environmental Allergology, Jagiellonian University Medical College, Krakow, Poland
- Nieves Prior, MD, PhD
- Allergy, Hospital Universitario Severo Ochoa, Madrid, Spain
- Avner Reshef, MD
- Angiedema Center, Barzilai University Medical Center, Ashkelon, Israel
- Marc Riedl, MD
- Division of Rheumatology, Allergy and Immunology, University of California San Diego, La Jolla, CA, USA
- Bruce Ritchie, MD
- Departments of Medicine and Medical Oncology, University of Alberta, Edmonton, AB, Canada
- Farrukh Rafique Sheikh, MBBS
- Section of Adult Allergy & Immunology, Department of Medicine, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia
- William B. Smith, MBBS, PhD
- Clinical Immunology and Allergy, Royal Adelaide Hospital, Adelaide, SA, Australia
- Peter J. Spaeth, PhD
- Institute of Pharmacology, University of Bern, Bern, Switzerland
- Marcin Stobiecki, MD
- Department of Clinical and Environmental Allergology, Jagiellonian University Medical College, Krakow, Poland
- Elias Toubi, MD
- Division of Allergy and Clinical Immunology, Bnai Zion Medical Center, Affiliated with Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel
- Lilian Agnes Varga, PhD
- Department of Internal Medicine and Haematology, Hungarian Angioedema Center of Reference and Excellence, Semmelweis University, Budapest, Hungary
- Karsten Weller, MD
- Institute of Allergology, Charité–Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.; Frauhofer Institute for Translational Medicine and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany
- Andrea Zanichelli, MD
- Department of Internal Medicine, ASST Fatebenefratelli Sacco, Ospedale Luigi Sacco-University of Milan, Milan, Italy
- Yuxiang Zhi, MD
- Department of Allergy and Clinical Immunology, Bejing Union Medical College Hospital, Chinese Academy of Medical Sciences, Bejing, China
- Bruce Zuraw, MD
- University of California, San Diego, San Diego, CA, United States
- Timothy Craig, MD
- Departments of Medicine and Pediatrics, Penn State University, Hershey, PA, USA
- Journal volume & issue
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Vol. 15,
no. 3
p. 100627
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.
