Management of hereditary angioedema: a Canadian approach

Area of Science:

• Immunology
• Genetics
• Rare Diseases

Background:

• C1 esterase inhibitor (C1-INH) deficiency is a rare genetic disorder.
• There is a lack of consensus on diagnosis, therapy, and management protocols for C1-INH deficiency.
• Canada's approach to C1-INH deficiency lags behind European standards.

Purpose of the Study:

• To establish the Canadian Hereditary Angioedema Society (CHAES)/Société d'angioédème héréditaire du Canada (SAHC).
• To foster knowledge and advance patient care for C1-INH deficiency in Canada.
• To present a review of current treatments and a proposed diagnostic and management algorithm for C1-INH deficiency in Canada.

Main Methods:

• Literature review of C1-INH deficiency treatments.
• Development of a diagnostic and management algorithm.
• Presentation of findings at the International Conference on Hereditary Angioedema.

Main Results:

• Formation of the Canadian Hereditary Angioedema Society (CHAES).
• Review of treatment strategies including prevention and replacement therapy.
• Proposal of a diagnostic, therapeutic, and management algorithm for C1-INH deficiency in Canada.

Conclusions:

• The establishment of CHAES aims to standardize and improve care for C1-INH deficiency in Canada.
• The proposed algorithm provides a framework for diagnosis and management.
• Further discussion and adoption of the algorithm are recommended to advance patient care.