Hereditary angioedema is a rare genetic disease that causes the rapid swelling of the limbs, face, intestinal tract, larynx or trachaea. The disease, which affects 1 in 50,000 people globally, is caused when a protein called C1 inhibitor is either deficient or non-functional. The symptoms of the disease cannot be controlled by conventional treatment with antihistamines or corticosteroids, and can lead to sudden death. The thematic series reviews the current international approach to the diagnosis, treatment and management of the disease. This includes investigating the management of the disease in children, which represents 50 % of clinical cases, and in women, who are more susceptible to the symptoms because of hormonal factors. The series also incorporates a comprehensive review of past, current and potential therapies for the disease.
The articles in the series were presented at the Toronto Consensus meeting organized by the Canadian Hereditary Angioedema Network, the Canadian Society of Allergy and Clinical Immunology and the University of Calgary. A final consensus document outlining the current global guidelines for the management of hereditary angioedema was agreed and authored by scientists who attended the meeting in Toronto.