Table 2 Targeted treatment of HAE [21, 63–66]
From: Angioedema in the emergency department: a practical guide to differential diagnosis and management
Drug | FDA-approved indication | Mechanism of action | Dose/route/price* | Time to onset of symptom relief | Adverse effects |
|---|---|---|---|---|---|
Plasma-derived C1-INH (Berinert) | Acute abdominal, facial, or laryngeal HAE attacks in adult and pediatric patients (no lower age limit established) | C1-INH protein replacement | 20 units/kg IV $8991 (1500 units) | Median: 48Â min | Common: dysgeusia Rare: anaphylaxis, thrombosis Theoretical: blood-borne infections |
Plasma-derived C1-INH (Cinryze) | Prophylaxis of HAE attacks in adults and pediatric patients ≥11 years of age | C1-INH protein replacement | 1000 units IV $5704 (1000 units) | Median: 30 min | Common: dysgeusia Rare: anaphylaxis, thrombosis Theoretical: blood-borne infections |
Recombinant C1-INH (Ruconest) | Acute HAE attacks in adults and pediatric patients ≥11 years of age; effectiveness not established for laryngeal attacks | C1-INH protein replacement | 50 units/kg IV $12,142 (4200 IU) | Median: 90 min | Common: sinusitis, rash, pruritus Rare: anaphylaxis |
Ecallantide (Kalbitor) | Acute HAE attacks in patients ≥12 years of age | Plasma-kallikrein inhibitor | 30 mg SC $14,090 (30 mg) | Median: 67 min | Common: headache, nausea, pyrexia, injection site reactions Uncommon: anaphylaxis (must be administered by a health care professional) |
Icatibant (Firazyr) | Acute HAE attacks in adults ≥18 years of age | Bradykinin-2 receptor antagonist | 30 mg SC $10,037 (30 mg) | Median: 2 h | Common: injection site reactions, pyrexia, increased transaminases, dizziness Theoretical: worsening of an ongoing ischemic event |