Choice of prophylactic agent for hereditary angioedema (HAE) in specific patient groups

Patient population	Preferred agents	Alternate agents	Agents to be avoided	Other notes
Prepubertal children (male and female)	Plasma-derived C1-INH*	Tranexamic acid (less effective but may be sufficient for mild disease) Lanadelumab	Androgens^¶	Berotralstat not studied or approved for children under 12 years of age
Adult females not considering pregnancy and postpubertal girls	Plasma-derived C1-INH* Lanadelumab Berotralstat	Tranexamic acid (less effective but may be sufficient for mild disease)	Androgens (multiple side effects and virilization)
Adult males and postpubertal boys	Plasma-derived C1-INH* Lanadelumab Berotralstat	Tranexamic acid (less effective but may be sufficient for mild disease) Androgens (multiple side effects but virilization less of an issue for men)
Pregnant and lactating people	Plasma-derived C1-INH* (has most safety data)	Tranexamic acid (less effective but history of safe use)	Androgens^Δ	Lanadelumab and berotralstat not recommended because they have not been studied in pregnancy

The choice of which long-term prophylactic agent to use is influenced both by patient characteristics (age, sex, pregnancy/lactation), as shown in the table, as well as regulatory requirements in different countries.

HAE: hereditary angioedema; C1-INH: C1 inhibitor.

* Plasma-derived C1-INH can be given subcutaneously or intravenously. Subcutaneous is more convenient and appears to be more effective based on preliminary evidence.

¶ Androgens are contraindicated in prepubertal children because they can cause premature closure of the growth plates.

Δ Androgens are avoided in pregnancy because they can result in virilization of female fetuses, although if an individual with HAE is carrying a male fetus, androgens have been successfully used with supervision by an endocrinologist.

Graphic 127119 Version 4.0

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Choice of prophylactic agent for hereditary angioedema (HAE) in specific patient groups