International Guideline on the Diagnosis and Management of Pediatric Patients With Hereditary Angioedema

Hereditary angioedema (HAE) with C1 inhibitor deficiency is a rare disease characterized by unpredictable episodes of tissue swelling (angioedema), which, in most cases, occur first under the age of 18 years, and entail a significant burden of disease not only for the patients but also for their families. Clinical symptoms of HAE are not specific, which may cause difficulties in differential diagnosis. Additionally, if not appropriately treated, HAE attacks can be life-threatening. The international HAE guidelines published so far have focused mainly on adults. A guideline that refers to the age-specific characteristics of pediatric patients, both in terms of diagnosis and management, was therefore needed. The International Steering Committee and Taskforce developed recommendations and provided evidence-based grading based on expert opinion and strength of evidence. Recommendations were presented to, discussed, and electronically voted by healthcare professionals during the 14th C1 Inhibitor Deficiency and Angioedema Workshop in Budapest, Hungary, 2025. This international guideline will ensure early diagnosis, standardized and up-to-date treatment, and promote the availability of effective therapies for all pediatric patients affected with this rare disease. It also draws attention to the importance of establishing HAE centers and registries, which solicit specialist care and research of the disease.

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Publication Details

Journal
Allergy
Published
2026-01-30
DOI
https://doi.org/10.1111/all.70207
Primary Topic
Coagulation, Bradykinin, Polyphosphates, and Angioedema
Type
article
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article

International Guideline on the Diagnosis and Management of Pediatric Patients With Hereditary Angioedema

K. Bork, Ankur Kumar Jindal, T. Craig, Dinh Van Nguyen et al.
Allergy
Coagulation, Bradykinin, Polyphosphates, and Angioedema
article

International Guideline on the Diagnosis and Management of Pediatric Patients With Hereditary Angioedema

K. Bork, Ankur Kumar Jindal, T. Craig, Dinh Van Nguyen, Andrea Luczay, Camelia Cristina Isaic, Hanga Réka Horváth, Anthony J. Castaldo, Bruce L. Zuraw, Henrik Balle Boysen, Sansanee Craig, Teresa Caballero, Avner Reshef, Mauro Cancian, Andrew MacGinnitie, Anastasios E Germenis, Markus Magerl, Grzegorz Porebski, Stephen Betschel, Anete S Grumach, Danny M Cohn, Inmaculada Martinez-Saguer, Francisco Contreras, Constance H Katelaris, Andrea Zanichelli, Hilary J Longhurst, Jonny Peter, Isabelle Boccon-Gibod, Henriette Farkas, the Hereditary Angioedema Working Group (HAWK Group)
article en

Abstract

Hereditary angioedema (HAE) with C1 inhibitor deficiency is a rare disease characterized by unpredictable episodes of tissue swelling (angioedema), which, in most cases, occur first under the age of 18 years, and entail a significant burden of disease not only for the patients but also for their families. Clinical symptoms of HAE are not specific, which may cause difficulties in differential diagnosis. Additionally, if not appropriately treated, HAE attacks can be life-threatening. The international HAE guidelines published so far have focused mainly on adults. A guideline that refers to the age-specific characteristics of pediatric patients, both in terms of diagnosis and management, was therefore needed. The International Steering Committee and Taskforce developed recommendations and provided evidence-based grading based on expert opinion and strength of evidence. Recommendations were presented to, discussed, and electronically voted by healthcare professionals during the 14th C1 Inhibitor Deficiency and Angioedema Workshop in Budapest, Hungary, 2025. This international guideline will ensure early diagnosis, standardized and up-to-date treatment, and promote the availability of effective therapies for all pediatric patients affected with this rare disease. It also draws attention to the importance of establishing HAE centers and registries, which solicit specialist care and research of the disease.

Allergy
Semmelweis University (HU), Goethe University Frankfurt (DE), Children's Hospital of Wisconsin (US), Jagiellonian University (PL), Camden and Campbelltown Hospitals (AU), La Jolla Institute for Immunology (US), United States Department of Veterans Affairs (US), Children's Hospital of Philadelphia (US), University of Padua (IT), University of Thessaly (GR), University of Cape Town (ZA), University of Toronto (CA), University of Milan (IT), Johannes Gutenberg University Mainz (DE), Medical College of Wisconsin (US), Hospital Universitario La Paz (ES), Auckland City Hospital (NZ), Groote Schuur Hospital (ZA), Hereditary Disease Foundation (US), Barzilai Medical Center (IL), University of California San Diego (US), Humboldt-Universität zu Berlin (DE), Tecnológico Nacional de México (MX), University Medical Center of the Johannes Gutenberg University Mainz (DE), IRCCS Policlinico San Donato (IT), VA San Diego Healthcare System (US), Hospital La Paz Institute for Health Research (ES), Fraunhofer Institute for Translational Medicine and Pharmacology (DE), Amsterdam Neuroscience (NL), Vinmec Times City International Hospital (VN), Bernstein Clinical Research Center (US), Laboratoire National de Référence (MA), University Hospital Frankfurt (DE), VinUniversity (VN), Faculdade de Medicina do ABC (BR), Manipal Hospital (IN), Amsterdam University of Applied Sciences (NL), Frankfurt School of Finance & Management (DE), University of Pennsylvania (US), Penn State Milton S. Hershey Medical Center (US), University of Amsterdam (NL), National Patient Safety Foundation (US), Université Grenoble Alpes (FR)
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Openalex Percentile: Top 72%
Coagulation, Bradykinin, Polyphosphates, and Angioedema
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