Hereditary angioedema: current and emerging treatment options

Abstract

Angioedema can result from allergic, hereditary, and acquired conditions. Hereditary angioedema (HAE) attacks are disabling at the time of occurrence and can be life threatening; they often result in hospitalization and intensive care unit admission. Although there are several variants of HAE, they share a final common pathway: unopposed activation of multiple kinins and mediators including kallikrein and bradykinin. This leads to increased vascular permeability, which in turn produces the edema after which the condition is named. Older treatment options licensed in the United States, anabolic steroids and antifibrinolytics, have troublesome side effect profiles and may not reverse a severe acute attack. In Europe, C1 esterase inhibitor (C1-INH) concentrates have been used since 1974 for both preventing and terminating attacks. Two of these have now been licensed in the United States for use in HAE patients, one for prophylaxis and the other for treating acute abdominal and facial HAE attacks. The first kinin pathway modulator, ecallantide, has also been licensed recently in the United States for treating HAE attacks. The objective of this article is to describe HAE and review the available options for managing patients, as well as different drugs currently under investigation. Specific attention is given to the perioperative management of patients with HAE.