The International/Canadian Hereditary Angioedema Guideline

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.

Primary Immunodeficiencies in Russia: Data From the National Registry

“…Though PAD are underrepresented, we have relatively large groups of autoinflammatory disorders (AID) and complement defects (predominantly HAE). This is because the Russian PID database collects data on all IUIS classified PIDs, in contrast with some other countries-where AID cases are followed and reported predominantly by rheumatologists, and HAE cases predominantly by allergists (23,24). In our registry, HAE patients contribute 12% of all PID cases and have a high rate of genetic confirmation, though diagnostic delay in these cases is still quite high.…”

Definition, aims, and implementation of GA2LEN/HAEi Angioedema Centers of Reference and Excellence

“…certified. It also provides (see Appendix S1) background information on GA 2 What are the requirements for GA 2 LEN/HAEi ACAREs? ACAREs are required to demonstrate excellence in the management of angioedema, research activities, efforts in education, and advocacy activity.…”

“…These guidelines and consensus documents include, for example, the international WAO/EAACI guideline for HAE, the EAACI/ GA 2 LEN/EDF/WAO guideline for urticaria, the International/ Canadian hereditary angioedema guideline, [2][3][4][5] Figure 1B).…”

The attack localizations in C1 inhibitor deficient hereditary angioedema patients

“…Due to the unpredictable and potentially fatal course screening. 21,22 Zanichelli et al stated that the C4 level was low in 96% of patients, and normal C4 values may eliminate the diagnosis of HAE-C1INH with an error probability of less than 0.05 . 15 According to a multicenter study, the combined use of antigenic C4 and C1INH function was described as a 98% specificity for HAE-C1INH with a negative predictive value of 96%.…”