c1-esterase inhibitor

C1-esterase inhibitor is a protein-based biologic therapy used to treat and prevent hereditary angioedema (HAE), a rare genetic disorder characterized by recurrent episodes of severe swelling in various parts of the body. The drug works by replacing or supplementing the deficient or dysfunctional endogenous C1-inhibitor, thereby regulating activation of the complement and contact system pathways. This action helps control inflammation and prevents excessive bradykinin production, which is responsible for increased vascular permeability and swelling during HAE attacks. There are both plasma-derived (Berinert, Cinryze, Haegarda) and recombinant (Ruconest) formulations available[2][4][5][6].

Brand names

BerinertCinryzeHaegardaRuconest

Other names

C1 esterase inhibitor (human)Human C1 esterase inhibitorPlasma protease C1 inhibitorComplement C1 esterase inhibitorC1-inhibiting factorC-1-inhibiting factorC 1-inhibiting factor