Icatibant as acute treatment for hereditary angioedema in adults

ABSTRACT

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.

KEYWORDS:

• Hereditary angioedema
• C1-inhibitor deficiency
• bradykinin
• bradykinin B2 receptor
• icatibant
• subcutaneous pre-filled injection
• self-administration
• acute treatment
• orphan drug

Acknowledgments

The author wishes to thank the patients for participating in the clinical studies, and thereby contributing to the efforts to make new therapeutic agents available in routine clinical practice. Further, the invaluable comments of Irmgard Andresen MD (Shire Pharmaceuticals) and the assistance of Nora Veszeli PhD student (3^rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary) in compiling the list of literature references is hereby acknowledged with sincere thanks.

Declaration of interests

H Farkas has received consultancy/speaker fees and honoraria from Shire, Swedish Orphan Biovitrum, Biocryst and CSL Behring. The author has no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed. No writing assistance was utilized in the production of this manuscript.