Update on therapeutic developments for hereditary angioedema

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Remarkable progress has been made in understanding the molecular mechanisms underlying attacks of swelling in hereditary angioedema (HAE). Treatment options in the United States for this potentially life-threatening disease had remained essentially static, however, over the past 40 years. Prophylactic therapy had relied on attenuated androgens or antifibrinolytic agents. Although demonstrably effective, these drugs have been fraught with side effects. Acute therapy has been largely relegated to supportive care. In this article we discuss emerging treatments that have evolved from the recognition that kinin generation is the fundamental abnormality leading to attacks of angioedema. We will review the newly approved replacement therapy for prophylaxis of HAE attacks with C1 inhibitor (C1INH). Potential options for the acute treatment of HAE will be discussed including purified C1INH, recombinant C1INH, an inhibitor of plasma kallikrein, and a B2-receptor antagonist. The arrival of these novel therapies promises to transform the future management of HAE.

Keywords: Angioedema; C1 inhibitor; antifibrinolytics; attenuated androgens; bradykinin; bradykinin B2 receptor; ecallantide; icatibant; prophylaxis

Document Type: Research Article

DOI: http://dx.doi.org/10.2500/aap.2009.30.3282

Affiliations: Department of Allergy, Kaiser Permanente and Department of Medicine, University of California San Diego, La Jolla, California, USA>. sandra.c.christiansen@kp.org

Publication date: September 1, 2009

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  • Allergy and Asthma Proceedings is a peer reviewed publication dedicated to distributing timely scientific research regarding advancements in the knowledge and practice of allergy, asthma and immunology. Its primary readership consists of allergists and pulmonologists.

    The goal of the Proceedings is to publish articles with a predominantly clinical focus which directly impact quality of care for patients with allergic disease and asthma.

    Featured topics include asthma, rhinitis, sinusitis, food allergies, allergic skin diseases, diagnostic techniques, allergens, and treatment modalities. Published material includes peer-reviewed original research, clinical trials and review articles.

    Articles marked "F" offer free full text for personal noncommercial use only.

    The journal is indexed in Thomson Reuters Web of Science and Science Citation Index Expanded, plus the National Library of Medicine's PubMed service.
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