期刊
EXPERT REVIEW OF CLINICAL PHARMACOLOGY
卷 9, 期 6, 页码 779-788出版社
TAYLOR & FRANCIS LTD
DOI: 10.1080/17512433.2016.1182425
关键词
Hereditary angioedema; C1-inhibitor deficiency; bradykinin; bradykinin B2 receptor; icatibant; subcutaneous pre-filled injection; self-administration; acute treatment; orphan drug
资金
- Shire
- Swedish Orphan Biovitrum
- Biocryst
- CSL Behring
Introduction: Hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) is a rare disease, characterized by recurrent, unpredictable episodes of cutaneous and/or mucosal edema. Bradykinin, released by the activation of the contact system, binds to bradykinin B2 receptors on the endothelial cell surface to enhance vascular permeaility, which leads to angioedema.Areas covered: C1-INH-HAE therapy is aimed at the inhibition of bradykinin release, as well as at the blockage of its effects mediated by its receptor. Three controlled trials, three open-label extensions, and two open-label studies, and a prospective, observational study have confirmed the safety and efficacy of the bradykinin B2 receptor antagonist, icatibant administered as acute treatment for HAE attacks in adult patients with C1-INH-HAE.Expert commentary: The ready-to-use, pre-filled syringes of icatibant can be self-administered easily, effectively, safely and, importantly, conviently. - This has resulted in patients being able to quickly treat an attack and realize a dramatic change for the better in their lives.
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