New promise and hope for treating hereditary angioedema.
BACKGROUND
While there is no approved effective therapy for the treatment of acute attacks of hereditary angioedema in the USA, four different drugs are completing or have recently completed Phase III clinical trials.


OBJECTIVE
To review the clinical status and future prospects of the new therapies under development for the treatment of hereditary angioedema.


METHODS
A review was carried out of the literature and presentations at meetings on the efficacy and safety of plasma-derived C1 inhibitor, recombinant human C1 inhibitor, the kallikrein inhibitor DX-88, and the B2 bradykinin receptor antagonist HOE-140.


CONCLUSIONS
Each of these drugs has been shown to be effective and safe for the treatment of hereditary angioedema; however, subtle differences in their mechanisms of action and delivery may influence how physicians and patients utilize the different drugs. The availability of effective therapy is expected to reshape the management of hereditary angioedema.
Zuraw
Bruce L
BL
Veterans Medical Research Foundation, University of California San Diego, La Jolla, CA 92093-0732, USA. bzuraw@ucsd.edu
Christiansen
Sandra C
SC
D054179:Angioedemas, Hereditary / Q000628:therapy*; D000818:Animals; D017326:Clinical Trials, Phase III as Topic; D003174:Complement C1 Inactivator Proteins / Q000008:administration & dosage / Q000172:deficiency / Q000627:therapeutic use; D050718:Complement C1 Inhibitor Protein; D006801:Humans; D010949:Plasma / Q000737:chemistry; D010951:Plasma Exchange; D011994:Recombinant Proteins / Q000008:administration & dosage / Q000627:therapeutic use; D015843:Serpins / Q000008:administration & dosage / Q000172:deficiency / Q000627:therapeutic use
D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; D011994:Recombinant Proteins; C469952:SERPING1 protein, human; D015843:Serpins