The International/Canadian Hereditary Angioedema Guideline
Creators: name: Betschel, Stephen
name: Badiou, Jacquie
name: Binkley, Karen
name: Borici-Mazi, Rozita
name: Hébert, Jacques
name: Kanani, Amin
name: Keith, Paul
name: Lacuesta, Gina
name: Waserman, Susan
name: Yang, Bill
name: Aygören-Pürsün, Emel
name: Bernstein, Jonathan
name: Bork, Konrad
name: Caballero, Teresa
name: Cicardi, Marco
name: Craig, Timothy
name: Farkas, Henriette
name: Grumach, Anete
name: Katelaris, Connie
name: Longhurst, Hilary
name: Riedl, Marc
name: Zuraw, Bruce
name: Berger, Magdelena
name: Boursiquot, Jean-Nicolas
name: Boysen, Henrik
name: Castaldo, Anthony
name: Chapdelaine, Hugo
name: Connors, Lori
name: Fu, Lisa
name: Goodyear, Dawn
name: Haynes, Alison
name: Kamra, Palinder
name: Kim, Harold
name: Lang-Robertson, Kelly
name: Leith, Eric
name: McCusker, Christine
name: Moote, Bill
name: O’Keefe, Andrew
name: Othman, Ibraheem
name: Poon, Man-Chiu
name: Ritchie, Bruce
name: St-Pierre, Charles
name: Stark, Donald
name: Tsai, Ellie
Publisher: name: BioMed Central
Year: 2019

description: 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.