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Self-administered C1 esterase inhibitor concentrates for the management of hereditary angioedema: usability and patient acceptance

Authors Li H

Received 4 February 2016

Accepted for publication 24 March 2016

Published 7 September 2016 Volume 2016:10 Pages 1727—1737


Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 4

Editor who approved publication: Dr Johnny Chen

Huamin Henry Li

Institute for Asthma and Allergy, Chevy Chase, MD, USA

Abstract: Hereditary angioedema (HAE) is a rare genetic disease characterized by episodic subcutaneous or submucosal swelling. The primary cause for the most common form of HAE is a deficiency in functional C1 esterase inhibitor (C1-INH). The swelling caused by HAE can be painful, disfiguring, and life-threatening. It reduces daily function and compromises the quality of life of affected individuals and their caregivers. Among different treatment strategies, replacement with C1-INH concentrates is employed for on-demand treatment of acute attacks and long-term prophylaxis. Three human plasma-derived C1-INH preparations are approved for HAE treatment in the US, the European Union, or both regions: Cinryze®, Berinert®, and Cetor®; however, only Cinryze is approved for long-term prophylaxis. Postmarketing studies have shown that home therapy (self-administered or administered by a caregiver) is a convenient and safe option preferred by many HAE patients. In this review, we summarize the role of self-administered plasma-derived C1-INH concentrate therapy with Cinryze at home in the prophylaxis of HAE.

Keywords: C1-INH concentrate, hereditary angioedema, disease management, first line, prophylaxis, self-administration

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