Current Allergy and Asthma Reports

, Volume 12, Issue 4, pp 273–280

Current Management Options for Hereditary Angioedema

ALLERGIC DERMATOSIS AND URTICARIA (J RING, SECTION EDITOR)

DOI: 10.1007/s11882-012-0273-4

Cite this article as:
Bork, K. Curr Allergy Asthma Rep (2012) 12: 273. doi:10.1007/s11882-012-0273-4

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 have been approved. 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 permitted for self-administration and home therapy. The number of management options has increased considerably within the last few years, thus helping to diminish the burden of HAE.

Keywords

Angioedema Hereditary angioedema Management C1 esterase inhibitor Bradykinin Kallikrein Androgens Danazol Treatment Prophylaxis Plasma-derived C1-INH Recombinant C1-INH Bradykinin B2 receptor antagonist Icatibant Conestat alpha Ecallantide Self-administration Home therapy Tranexamic acid 

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  1. 1.Department of DermatologyJohannes Gutenberg UniversityMainzGermany

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