Table 1.

Current treatment options for hereditary angioedema with C1-inhibitor deficiency

Drug (trade name)	Manufacturer	Mechanism of action	Indication	Administration	Age indications
Attenuated androgens: danazol, oxandrolone, and stanozolol	Generic manufacturers	AA induce aminopeptidase P activity and increase C1-INH synthesis and C1-INH mRNA expression	LTP, STP	Oral	≥18 years
Berotralstat (Orladeyo®)	BioCryst Pharmaceuticals	Kallikrein inhibitor	LTP	Oral	FDA:≥12 years, under review for EMA approval
Plasma-derived C1-INH (Berinert®)	CSL Behring	Plasma-derived C1-INH concentrate	ODT, STP	Intravenous	All
Plasma-derived C1-INH (Cinryze®)	Takeda	Plasma-derived C1-INH concentrate	LTP, ODT, STP	Intravenous	LTP ≥6 years, ODT and STP ≥2 years
Conestat alfa (Ruconest®)	Pharming Group NV	Recombinant C1-INH concentrate	ODT	Intravenous	FDA: adolescents and adults EMA: ≥2 years
Ecallantide (Kalbitor®)	Takeda	Kallikrein inhibitor	ODT	Subcutaneous, no self-administration	≥12 years
Plasma-derived C1-INH (Haegarda®)	CSL Behring	Plasma-derived C1-INH concentrate	LTP	Subcutaneous	≥12 years
Icatibant (Firazyr®)	Takeda	BKRB2 antagonist	ODT	Subcutaneous	FDA: ≥18 years EMA: ≥2 years
Lanadelumab (Takhzyro®)	Takeda	Kallikrein inhibitor	LTP	Subcutaneous	≥12 years
Tranexamic acid (Cyklokapron®)	Generic manufacturers	Competitive inhibitor of plasminogen-mediated FXII activation	LTP	Oral	Adolescents and adults

AA attenuated androgens, BKRB2 bradykinin receptor B2, C1-INH C1 esterase inhibitor, LTP long-term prophylaxis, ODT on demand treatment, STP short-term prophylaxis