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Abstract: Poster Presentations |

ANGIOEDEMA: THERAPEUTIC OPTIONS TO SUIT THE PATHOPHYSIOLOGY: AN UPDATE ON HEREDITARY ANGIOEDEMA (HAE) FREE TO VIEW

Ira Kalfus, MD*; Glenn S. Tillotson, PhD
Author and Funding Information

M2G Consulting, New York, NY


Chest


Chest. 2009;136(4_MeetingAbstracts):42S-b-43S. doi:10.1378/chest.136.4_MeetingAbstracts.42S-b
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Abstract

PURPOSE:  Describe current and new approaches to managing angioedema.

METHODS:  Angioedema is a constellation of syndromes characterized by swelling of deep layers of skin or submucosa, possibly both. The causes of angioedema are multiple and are often categorized as being drug-induced, idiopathic, acquired, and hereditary. The underlying mechanisms are different and warrant appropriate therapeutic management. The majority of angioedema episodes respond to anti-histamines or corticosteroids although these approaches are of no value in HAE. HAE is an autosomal dominant inherited disorder that arises from a deficiency of functional C1 esterase inhibitor (C1-INH). C1-INH is a SERPIN which acts as a suicide inhibitor enzyme critical to the control of three major pathways; complement, contact and fibrinolytic. These systems collectively affect inflammatory processes to eradicate pathogens or maintain coagulation. Triggers of HAE attacks include hormonal influence, repetitive motion, stress etc. The treatment options available for HAE have changed. Until recently the only approved preventative treatment of HAE in the US was attenuated androgens, and danazol is most commonly used. However these agents are associated with significant side effects especially in women and children. C1-INH, CinryzeÖ (ViroPharma Incorporated, Exton, PA) was approved by FDA in October 2008 for prophylactic use. Two randomized, controlled studies were conducted to establish the efficacy and safety of this human plasma-derived, nanofiltered C1 esterase inhibitor therapy.

RESULTS:  22 patients were evaluated in a randomized, double-blind, placebo-controlled, cross-over study of the prevention of attacks of HAE. C1 INH, Cinryze™ was shown to significantly reduce the number of attacks, intensity of attacks and duration of attacks. It was also well tolerated. 68 patients were treated with Cinryze™ for acute attacks of HAE.

CONCLUSION:  C1 INH, Cinryze™ was shown to significantly reduce the number of attacks, the intensity of attacks and duration of attacks. It was also well tolerated. 68 patients were treated with Cinryze™ for acute attacks of HAE.

CLINICAL IMPLICATIONS:  Cinryze™ was shown to be efficacious and well-tolerated for acute attacks and the prevention of attacks in patients with HAE.

DISCLOSURE:  Ira Kalfus, Employee Glenn Tillotson is a full-time employee of ViroPharma Incorporated, he holds no stock of the company.; Consultant fee, speaker bureau, advisory committee, etc. Ira Kalfus is a part-time consultant to ViroPharma Incorporated, he holds stock in ViroPharma.; No Product/Research Disclosure Information

Tuesday, November 3, 2009

12:45 PM - 2:00 PM


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