Table 1 Drugs Commonly Used for Acute and Prophylactic Treatment of HAE[2, 9, 25, 45–48]
Drug Class or Name | Adult Dosage and Route of Administration | Mechanism of Action |
|---|---|---|
Acute therapy | Â | Â |
   C1-esterase inhibitor (human) | 20 U/kg IV | Replaces missing or malfunctioning C1-esterase inhibitor |
   Ecallantide | 30 mg SC split into 3 injections | Potent, selective, reversible inhibitor of plasma kallikrein, which reduces the conversion of high-molecular-weight kininogen to bradykinin |
   Icatibant | 30 mg SC | Selective competitive bradykinin type 2 receptor antagonist |
Prophylactic therapy | ||
17-alpha alkylated androgens | Â | Exact mechanism not known. Thought to increase endogenous C1-esterase inhibitor levels via hepatic synthesis and a subsequent increase in the expression of mRNA |
   Danazol | 100 mg PO every 3 days to 600 mg QD |  |
   Oxandrolone | 2.5 mg PO every 3 days to 20 mg QD |  |
   Stanozolol | 1 mg PO every 3 days to 6 mg QD |  |
Antifibrinolytics | Â | Inhibit the formation and activity of plasmin and subsequently decrease plasmin-induced activation of C1 |
   Tranexamic acid | 20-50 mg/kg/d PO split BID or TID |  |
   ε-aminocaproic acid | 8 to 12 g PO daily in 4 divided doses |  |
Nanofiltered C1-esterase inhibitor (human) | 1000 U IV every 3 to 4 days | Replaces missing or malfunctioning C1-esterase inhibitor |