Hereditary angioedema (HAE) is due to a genetic C1 inhibitor deficiency and presents with recurrent skin swellings, episodic abdominal pain, and potentially life-threatening laryngeal edema. The underlying vasodilation and vasopermeability are mediated by bradykinin.
As of 2007, a plasma pool C1-esterase inhibitor (C1-INH) concentrate is the therapy of choice in HAE. Several new drugs have been developed for treatment of angioedema: a recombinant C1-INH, a kallikrein inhibitor (ecallantide) and a specific bradykinin-B2-receptor antagonist (icatibant).
Icatibant is a bradykinin receptor-2 antagonist which seemed beneficial in a small study involving 15 patients.
Icatibant, a bradykinin receptor-2 antagonist, for treatment of hereditary angioedema.
JACI, The Editors' Choice, Volume 119, Issue 6, Pages 1289-1290 (June 2007). Click for the original article.
Nonallergic angioedema: role of bradykinin. M. Bas, V. Adams, T. Suvorava, T. Niehues, T. K. Hoffmann, G. Kojda (2007). Allergy 62 (8), 842–856.
Escallantide, an Inhibitor of Kallikrein Acts Against Hereditary Angioedema. Medscape, 2007.