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Update on the acute treatment of hereditary angioedema

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Hereditary angioedema (HAE) is a genetic autosomal dominant condition caused by C1-esterase inhibitor protein (C1INH) deficiency that results in episodic tissue angioedema. Recently, new therapies have been developed to more effectively manage this rare but serious condition. This review will provide a concise summary of HAE acute treatment options for the practicing allergist/immunologist. Clinical study data for emerging HAE therapies were reviewed and summarized. Based on efficacy and safety data from completed clinical studies, three new HAE treatments have recently been approved by the Food and Drug Administration: nanofiltered plasma-derived C1INH for prophylactic therapy, pasteurized plasma-derived C1INH for acute therapy, and ecallantide for acute therapy. Two other promising therapies, recombinant C1INH and icatibant, are in various stages of the U.S. regulatory process. The medical management of HAE is entering a new era with the availability of safe, effective condition-specific treatments. Clinicians should consider a number of patient- and medication-specific factors when designing individualized treatment plans for HAE patients.

Keywords: Acute treatment; C1 inhibitor; C1-INH replacement products; bradykinin B2-receptor antagonist; clinical studies; ecallantide; hereditary angioedema; icatibant; individualized therapy

Document Type: Research Article

Affiliations: Section of Clinical Immunology and Allergy, Department of Medicine, University of California–Los Angeles, David Geffen School of Medicine, Los Angeles, California, USA

Publication date: 01 January 2011

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