Approximately 30,000 individuals in the U.S. have Hereditary Angioedema (HAE).
Icatibant, a bradykinin B2 receptor antagonist, is supplied in a prefilled syringe. Itc can be stored at room temperature (up to 77°F) for immediate use.
New therapies for hereditary angioedema (HAE) (click to enlarge the image).
Two other agents were approved by the FDA in 2009 for treatment of HAE attacks:
- plasma-derived C1-esterase inhibitor concentrate for injection (Berinert) for HAE-related acute abdominal attacks and facial swelling. HAE patients can self-administer Berinert C1-INH by IV infusion - FDA, 2012.
- ecallantide subcutaneous injection (Kalbitor) for treatment of HAE attacks
Cinryze (C1-esterase inhibitor concentrate) is approved for prophylaxis of HAE, not for treatment of acute attacks.
This is a good summary from Medscape's Atopic Topics blog:
Unlike Ecallantide which is only approved for administration in a supervised setting (due to risk of hypersensitivity reaction), Firazyr is approved for use by the patient.
Screening for HAE is a simple process - C4 is always low in HAE (unless the patient is already treated for the disease). A normal C4 therefore rules it out. If C4 is low, a quantitative AND qualitative C1 inhibitor levels should be checked
FDA Approves New Drug for Hereditary Angioedema. Medscape, 2011.
HAE patients can self-administer Berinert C1-INH by IV infusion - FDA, 2012.