. 2019 Aug 1;7(1):75–83. doi: 10.1016/j.gendis.2019.07.002

Table 2.

Summary of effective treatment options for hereditary angioedema.

Therapeutic options	Uses	Suggested doses	Remarks
Danazol Stanozolol Oxandrolone (Weak androgens)	Short term & Long term prophylaxis	Danazol - STP: 2.5–10 mg/kg/day (maximum 600 mg/day). LTP: 2.5–5 mg/kg/day (maximum 200 mg/day).	Easily available. Undesirable virilising effect, weight gain, acne, headaches, menstrual irregularities, depression.
Tranexamic acid Epsilon aminocaproic acid	Short term & Long term prophylaxis	Tranexamic acid: 20–50 mg/kg/day in 2 or 3 divided doses (maximum 3–6 g/day).	Can be used in pregnancy if benefits outweigh risk (category B)
FFP (fresh frozen plasma)/ SDP (solvent detergent plasma)	Short term prophylaxis Acute treatment	10 ml/kg/dose	Easily available, inexpensive. Concern of infections with regular use.
C1 esterase inhibitors	pdC1-INH_Be for short term prophylaxis, acute treatment	pdC1-INH_Be 15–30 units/kg/dose IV.	Expensive, currently unavailable in many Asian countries. One unit parallels the amount of C1INH in 1 ml of plasma of a normal individual.
	pdC1-INH_Ci for long term prophylaxis, acute treatment	pdC1-INH_Ci 10–20 units/kg/dose once or twice weekly (initial maximum dose 1000 units).
	rhC1-INH for acute treatment, long term prophylaxis	rhC1-INH 50 units/kg/dose and is given by intravenous injection.
	rhC1-INH for acute treatment, long term prophylaxis	Subcutaneous preparations are also available.
Ecallantide	Acute treatment	30 mg/dose subcutaneous injection	Expensive, risk of anaphylaxis. Not available in many Asian countries.
Icatibant	Acute treatment	30 mg/dose subcutaneous injection (maximum three doses/day)	Expensive. Not available in many Asian countries. Local reactions.
Lanadelumab	Long term prophylaxis	Dose range 150–300 mg every 2–4 weeks.	Pediatric dosing yet to be established.
BCX7353	Long term prophylaxis	Minimum effective dose used: 125 mg/day	Pediatric dosing yet to be established.