Table 1 On-demand and prophylaxis medications approved for HAE-C1-INH in the United States [9]
Brand name (Generic) | Class | Ages | Dosage | Route (self-administration) | Adverse effects |
|---|---|---|---|---|---|
On-demand therapy | |||||
 Berinert (C1-INH) [46] | Plasma-derived C1-inhibitor | Pediatric and adults of all ages | 20 U/kg | IV (yes) | Allergic reaction, nausea, diarrhea |
 Ruconest (C1-INH) [47] | Recombinant C1-inhibitor | Adolescents and adults | 50 U/kg | IV (yes) | Headache, nausea, diarrhea |
 Kalbitor (ecallantide) [25] | Kallikrein inhibitor |  ≥ 12 years | 30 mg | SQ (no) | Possible anaphylaxis (uncommon) |
 Firazyr (icatibant) [26] | B2 bradykinin receptor antagonist |  ≥ 18 years | 30 mg | SQ (yes) | Redness, swelling, pain at the site of injection |
Prophylaxis | |||||
 Danazol [39] | Attenuated androgen |  ≥ 18 years | Variable, with maximum long-term recommended dosage of 200 mg/day | Oral | Weight gain, virilization, hirsutism, acne, voice changes (hoarseness/deepening), menstrual irregularities, pseudomenopause, vaginal burning, vaginal dryness or itching, hypercholesterolemia, hypertension, hepatotoxicity |
 Cinryze (C1-INH) [48] | Plasma-derived C1-inhibitor |  ≥ 6 years | 500 IU in children 6–11 years, 1000–2500 IU in adults, twice weekly | IV (yes) | Allergic reaction, nausea, diarrhea |
 Haegarda (C1-INH) [49] | Plasma-derived C1-inhibitor |  ≥ 6 years | 60 IU/kg twice weekly | SQ (yes) | Injection site reactions |
 Takhzyro (lanadelumab-flyo) [41] | Plasma kallikrein inhibitor |  ≥ 12 years | 300 mg every 2 weeks initially; may be given every 4 weeks based on clinical response | SQ (yes) | Injection site reactions, upper respiratory infections, headache |
 Orladeyo (berotralstat) [42] | Plasma kallikrein inhibitor |  ≥ 12 years | 150 mg daily | Oral | Abdominal pain, vomiting, diarrhea, back pain, gastroesophageal reflux |