The International/Canadian Hereditary Angioedema Guideline
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Published:2019-11-25
Issue:1
Volume:15
Page:
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ISSN:1710-1492
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Container-title:Allergy, Asthma & Clinical Immunology
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language:en
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Short-container-title:Allergy Asthma Clin Immunol
Author:
Betschel StephenORCID, Badiou Jacquie, Binkley Karen, Borici-Mazi Rozita, Hébert Jacques, Kanani Amin, Keith Paul, Lacuesta Gina, Waserman Susan, Yang Bill, Aygören-Pürsün Emel, Bernstein Jonathan, Bork Konrad, Caballero Teresa, Cicardi Marco, Craig Timothy, Farkas Henriette, Grumach Anete, Katelaris Connie, Longhurst Hilary, Riedl Marc, Zuraw Bruce, Berger Magdelena, Boursiquot Jean-Nicolas, Boysen Henrik, Castaldo Anthony, Chapdelaine Hugo, Connors Lori, Fu Lisa, Goodyear Dawn, Haynes Alison, Kamra Palinder, Kim Harold, Lang-Robertson Kelly, Leith Eric, McCusker Christine, Moote Bill, O’Keefe Andrew, Othman Ibraheem, Poon Man-Chiu, Ritchie Bruce, St-Pierre Charles, Stark Donald, Tsai Ellie
Abstract
AbstractThis 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.
Publisher
Springer Science and Business Media LLC
Subject
Pulmonary and Respiratory Medicine,Immunology,Immunology and Allergy
Reference171 articles.
1. Bork K, Hardt J, Witzke G. Fatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency. J Allergy Clin Immunol. 2012;130(3):692–7. 2. Agostoni A, Aygören-Pürsün E, Binkley KE, Blanch A, Bork K, Bouillet L, et al. Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J Allergy Clin Immunol. 2004;114(3):S51–131. 3. Zilberberg MD, Jacobsen T, Tillotson G. The burden of hospitalizations and emergency department visits with hereditary angioedema and angioedema in the United States, 2007. 2010. http://www.ingentaconnect.com/content/ocean/aap/2010/00000031/00000006/art00014. Accessed 20 Mar 2019. 4. Cicardi M, Agostoni A. Hereditary Angioedema. N Engl J Med. 1996;334(25):1666–7. 5. Zuraw BL. Hereditary angioedema. N Engl J Med. 2008;359(10):1027–36.
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