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.

Hereditary angioedema (HAE) is a disease which is associated with 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 is neither optimal nor uniform across the country. It lags behind other countries where there are more organized models for HAE management, and where additional therapeutic options are licensed and available for use. The objective of this guideline is to provide graded recommendations for the management of patients in Canada 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. It is anticipated that by providing this guideline to caregivers, policy makers, patients and their advocates, that there will be an improved understanding of the current recommendations regarding management of HAE and the factors that need to be considered when choosing therapies and treatment plans for individual patients. 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 physicians, gastroenterologists, dentists and otolaryngologists, 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.Electronic supplementary materialThe online version of this article (doi:10.1186/1710-1492-10-50) contains supplementary material, which is available to authorized users.

An amendment to this paper has been published and can be accessed via the original article.

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Introduction: Hereditary angioedema (HAE) patients (both diagnosed and undiagnosed) commonly present to the emergency department (ED). Presenting symptoms (swelling and pain) may be erroneously attributed to common allergic and gastrointestinal conditions resulting in major delays in diagnosis and appropriate treatment. No published tools currently exist for HAE screening and management in undiagnosed disease. The overall goal of the study was to develop a HAE-RT tool for ED settings. Methods: A two-phase mixed methods approach was used to develop the HAE-RT Tool including: Phase 1: A Delphi Study [HAE specialists (N=9) and National Patient Advocacy Group Members (N=3)] was conducted to reach consensus (80% agreement) on predictor variables to include. Phase 2: A retrospective chart review was conducted to assess the predictive findings of the predictor variables. A convenient sample of patients presenting with angioedema (with and without HAE) between January 2012 January 2017 were included in the study. Results: Of the 12 experts invited, 9 (75%) participated in the Delphi study. Of 8 HAE-specific predictive variables, 4 reached consensuses including: (1) recurrent angioedema; (2) absence of urticaria; (3) past recurrent abdominal pain/swelling; (4) response to allergic therapy. The retrospective study included 85 patients (N=46 with HAE; N=39 non-HAE; overall 72% female). HAE patients were significantly more likely to have a family history of HAE (72% vs 0%; P<0.0001); previous recurrent angioedema (96%; P<0.009); present with no hives (91%; P<0.036); previous recurrent abdominal pain (80%; P<0.0001); and only 2% responded positively to allergy treatments (P<0.0001). Conclusion: Our study emphasizes the importance of key stakeholder involvement and feedback to facilitate the prioritization of important information that must be included in the design of an HAE-RT tool. The next step is to observe the effect of the HAE-RT tool on patient triage in the ED.

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RATIONALE: Hereditary Angioedema (HAE) is a complex debilitating disease that is often misdiagnosed and under treated. Our study objective was to gain insight into which treatments Canadians are using for their acute attacks, prophylactic treatments, and the frequency of administration. METHODS: In 2017-2018, the first National Canadian HAE survey was electronically sent to all HAE Canada members. The following data was based solely on adult participants. RESULTS: Participants are treating with: C1 esterase Inhibitor (Berinert)-61%, C1 esterase Inhibitor (Cinryze)-3%, Icatibant (Firazyr)-10%, Androgen (Danazol)-5%, Tranexamic acid-3%, No pharmaceutical treatment-3%, and Other-15%. Berinert is being used: On demand (36%), Chronic Prophylaxis only (9%), Chronic Prophylaxis and on demand (55%). Cinryze is being administered for: Chronic Prophylaxis only (33%), and for both Chronic Prophylaxis and on demand (67%). Firazyr is being administered: On demand (89%), and for both Chronic Prophylaxis and on demand (11%). Danazol is being used for: On demand (25%), Chronic Prophylaxis only (25%), Chronic Prophylaxis and on demand (50%). Tranexamic acid is taken for Chronic Prophylaxis and on demand (100%) by all respondents. CONCLUSIONS: The data collected demonstrates that treatments are being used interchangeably for acute and prophylaxis treatment despite the indications listed on the product monographs. These results validate that patients in consultation with their HAE specialists have determined an individualized treatment schedule. All results are limited to the respondents and may not represent the broader Canadian HAE population.