The International/Canadian Hereditary Angioedema Guideline

Stephen Betschel, Jacquie Badiou, Karen Binkley, Rozita Borici-Mazi, Jacques Hébert, Amin Kanani, Paul Keith, Gina Lacuesta, Susan Waserman, Bill Yang, Emel Aygören-Pürsün, Jonathan Bernstein, Konrad Bork, Teresa Caballero, Marco Cicardi, Timothy Craig, Henriette Farkas, Anete Grumach, Connie Katelaris, Hilary LonghurstMarc Riedl, Bruce Zuraw, Magdelena Berger, Jean Nicolas Boursiquot, Henrik Boysen, Anthony Castaldo, Hugo Chapdelaine, Lori Connors, Lisa Fu, Dawn Goodyear, Alison Haynes, Palinder Kamra, Harold Kim, Kelly Lang-Robertson, Eric Leith, Christine McCusker, Bill Moote, Andrew O'Keefe, Ibraheem Othman, Man Chiu Poon, Bruce Ritchie, Charles St-Pierre, Donald Stark, Ellie Tsai

Research output: Contribution to journalReview articlepeer-review

95 Scopus citations


This is an update to the 2014 Canadian Hereditary Angioedema Guideline with an expanded scope to include the management of hereditary angioedema (HAE) patients worldwide. It is a collaboration of Canadian and international HAE experts and patient groups led by the Canadian Hereditary Angioedema Network. The objective of this guideline is to provide evidence-based recommendations, using the GRADE system, for the management of patients with HAE. This includes the treatment of attacks, short-term prophylaxis, long-term prophylaxis, and recommendations for self-administration, individualized therapy, quality of life, and comprehensive care. New to the 2019 version of this guideline are sections covering the diagnosis and recommended therapies for acute treatment in HAE patients with normal C1-INH, as well as sections on pregnant and paediatric patients, patient associations and an HAE registry. Hereditary angioedema results in random and often unpredictable attacks of painful swelling typically affecting the extremities, bowel mucosa, genitals, face and upper airway. Attacks are associated with significant functional impairment, decreased health-related quality of life, and mortality in the case of laryngeal attacks. Caring for patients with HAE can be challenging due to the complexity of this disease. The care of patients with HAE in Canada, as in many countries, continues to be neither optimal nor uniform. It lags behind some other countries where there are more organized models for HAE management, and greater availability of additional licensed therapeutic options. It is anticipated that providing this guideline to caregivers, policy makers, patients, and advocates will not only optimize the management of HAE, but also promote the importance of individualized care. The primary target users of this guideline are healthcare providers who are managing patients with HAE. Other healthcare providers who may use this guideline are emergency and intensive care physicians, primary care physicians, gastroenterologists, dentists, otolaryngologists, paediatricians, and gynaecologists who will encounter patients with HAE and need to be aware of this condition. Hospital administrators, insurers and policy makers may also find this guideline helpful.

Original languageEnglish (US)
Article number72
JournalAllergy, Asthma and Clinical Immunology
Issue number1
StatePublished - Nov 25 2019

All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Immunology
  • Pulmonary and Respiratory Medicine


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