Current management options for hereditary angioedema.
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.
Department of Dermatology, Johannes Gutenberg University, Mainz, Germany. bork@hautklinik.klinik.uni-mainz.de
Konrad
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Bork
D054179:Angioedemas, Hereditary / Q000188:drug therapy*; D001920:Bradykinin / Q000031:analogs & derivatives / Q000627:therapeutic use; D050718:Complement C1 Inhibitor Protein / Q000627:therapeutic use; D003613:Danazol / Q000627:therapeutic use; D019468:Disease Management; D006801:Humans; D010074:Oxandrolone / Q000627:therapeutic use; D010455:Peptides / Q000627:therapeutic use; D010949:Plasma; D011994:Recombinant Proteins / Q000627:therapeutic use; D013197:Stanozolol / Q000627:therapeutic use
D050718:Complement C1 Inhibitor Protein; D010455:Peptides; D011994:Recombinant Proteins; D013197:Stanozolol; C511194:ecallantide; C571093:conestat alfa; D010074:Oxandrolone; C065679:icatibant; D003613:Danazol; D001920:Bradykinin