Recent advances in the management of hereditary angioedema.
Publication/Presentation Date
7-1-2013
Abstract
Hereditary angioedema (HAE) is a rare genetic condition that manifests as painful and potentially life-threatening episodic attacks of cutaneous and submucosal swelling. It results from functional deficiency of C1 inhibitor (C1 INH), which is a regulator of the complement, fibrinolytic, kinin (contact), and coagulation systems. In patients with HAE, the low plasma concentration of functional C1 INH leads to overactivation of the kinin cascade and local release of bradykinin. Bradykinin is responsible for the pain, vascular permeability changes, and edema associated with HAE. Until recently, therapeutic options for HAE have been very limited. Many new therapies have emerged, however, such as C1 INH replacement drugs and medications aimed at components of the contact system (eg, plasma kallikrein inhibitor and bradykinin B2 receptor antagonist). The authors review current and novel treatments for patients with HAE.
Volume
113
Issue
7
First Page
546
Last Page
555
ISSN
1945-1997
Published In/Presented At
Hemperly, S. E., Agarwal, N. S., Xu, Y. Y., Zhi, Y. X., & Craig, T. J. (2013). Recent advances in the management of hereditary angioedema. The Journal of the American Osteopathic Association, 113(7), 546–555. https://doi.org/10.7556/jaoa.2013.006
Disciplines
Medicine and Health Sciences
PubMedID
23843378
Department(s)
Department of Medicine
Document Type
Article