Abstract
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.
Highlights
Hereditary angioedema (HAE) results in random and often unpredictable attacks of painful swelling typically affecting the extremities, bowel mucosa, genitals, face and upper airway [1]
Androgens and anti-fibrinolytics are not recommended as first line, these agents may be considered for Long-term prophylaxis (LTP) in those patients who have already obtained benefit from their use or who have difficulty obtaining first-line options
All patients should be encouraged to join their local and international patient organizations. This update to the 2014 Canadian Hereditary Angioedema Guideline is the collaborative effort of Canadian and international hereditary angioedema (HAE) experts and patient groups led by the Canadian Hereditary Angioedema Network
Summary
Hereditary angioedema (HAE) results in random and often unpredictable attacks of painful swelling typically affecting the extremities, bowel mucosa, genitals, face and upper airway [1]. Attacks are associated with significant functional impairment, decreased healthrelated quality of life (HRQoL), and mortality in the case of laryngeal attacks [2, 3]. HAE-1 is the most prevalent, representing approximately 85% of cases, and results from low antigenic and functional levels of C1-INH. HAE-2 accounts for approximately 15% of cases and is associated with a normal C1-INH protein concentration but impaired C1-INH function [6, 7]. The swelling in HAE-1/2 is a result of impaired regulation of bradykinin synthesis [8]. Bradykinin is a very powerful vasodilator that increases capillary permeability, constricts smooth muscle, and stimulates pain receptors [4, 5]
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