A new oral kallikrein inhibitor for long-term prophylaxis of hereditary angioedema.

Abstract

Hereditary angioedema (HAE) is a rare inherited chronic disease characterized by unpredictable recurrent attacks of cutaneous and submucosal swelling potentially involving airways, frequently upper ones, and compromising patient's life. This implies a serious health problem with significant disease burden and reduced life quality. This disease presents a great clinical variability and different endotypes, hampering diagnosis and requiring a personalized treatment. The primary mediator of swelling in most phenotypes is bradykinin, which increases vasodilation and vascular permeability by binding to the bradykinin B2 receptor.