From MedPage Today:
Between 8,000 and 10,000 people in the U.S. are affected by the most common form of HAE which is caused by an autosomal dominant mutation that decreases C1 inhibitor levels.
Plasma kallikrein plays a major role in the contact (kallikrein-kinin) cascade producing bradykinin. Bradykinin is a vasodilator, which increases vascular permeability, activates inflammation and produces pain.
New therapies for hereditary angioedema (HAE) (click to enlarge the image).
Ecallantide (DX-88) is a subcutaneous kallikrein inhibitor which is under investigation for treatment of hereditary angioedema (HAE).
In the clinical trials, the proportion of patients who said they had complete or near-complete resolution varied by attack site:
- For abdominal attacks, 65.2% of those on the drug reported such an outcome, compared with 31.7% for those on placebo (P=0.01).
- For laryngeal attacks, the comparable proportion were 60% versus 11.1% (P=0.03).
- For peripheral attacks, there was no significant difference within the first four hours.
However, the median times -- 67 minutes for the drug versus 105 minutes for placebo -- were not significantly different.
ACAAI: HAE Drug Offers Faster Relief. Michael Smith, North American Correspondent, MedPage Today.
Ecallantide (DX-88), a plasma kallikrein inhibitor for the treatment of hereditary angioedema and the prevention of blood loss in on-pump cardiothoracic surgery. Lehmann A. Expert Opin Biol Ther. 2008 Aug;8(8):1187-99.
Virtually No Relapses After Ecallantide for Acute HAE attacks, despite short half-life. Medscape, 2011.