Abstract

The aim of treatment of hereditary angioedema (HAE) due to C1 esterase inhibitor deficiency (HAE-C1-INH) is either treating acute attacks or preventing attacks by using prophylactic treatment. For treating acute attacks, plasma-derived C1 inhibitor (C1-INH) concentrates, a bradykinin B2 receptor antagonist, and a recombinant human C1-INH are available in Europe. In the United States, a plasma-derived C1-INH concentrate, a bradykinin B2 receptor antagonist, and a plasma kallikrein inhibitor were approved for the treatment of acute attacks. Fresh frozen plasma is also available for treating acute attacks. Short-term prophylactic treatment focuses on C1-INH and attenuated androgens. Long-term prophylactic treatments include attenuated androgens such as danazol, stanozolol, and oxandrolone, antifibrinolytics, and a plasma-derived C1-INH concentrate. Plasma-derived C1-INH and a bradykinin B2 receptor antagonist are admitted for selfadministration and home therapy. So the number of management options increased considerably within the last few years thus helping to diminish the burden of HAE.

German version published in Allergologie, Vol. 36, No. 3/2013, pp. 108-119

Author Details

Authors

• K. Bork

Departments

• Universitäts-Hautklinik, Johannes-Gutenberg-Universität, Mainz

Address

Univ.-Prof. Dr. med. K. Bork
Universitäts-Hautklinik
Johannes-Gutenberg-Universität
Langenbeckstraße 1
D-55131 Mainz
Email: [email protected]

Citation