Complement C1 Esterase Inhibitor

Synonyms: Inhibidor de la C1 esterasa.
Cyrillic synonym: С1-Ингибитор Комплемента.

💊 Chemical information

ATC — B02AB03.
ATC Vet — QB02AB03.

💊 Profile

Complement C1 esterase inhibitor is an endogenous complement blocker that plays a role in regulation of the complement system. It is prepared from human plasma and given as replacement therapy in hereditary angioedema, in which there is a deficiency of natural complement C1-esterase inhibitor. It is given for both short-term prophylaxis and treatment of acute life-threatening attacks by slow intravenous injection or infusion in typical doses of 500 units or, in severe cases, 1000 units. The dose may be repeated if necessary after a few hours. A recombinant human complement C1 esterase inhibitor (rh C1INH) is under investigation. It has also been tried in the management of other conditions including sepsis and capillary leak syndrome.2 It is under investigation for the treatment of pancreatitis and for use in allogeneic lung transplantation, thermal injury, and shock.2 It is also being studied as a means of limiting reperfusion injury in patients with acute myocardial infarction.3
1. Waytes AT, et al. Treatment of hereditary angioedema with a vapor-heated C1 inhibitor concentrate. N Engl J Med 1996; 334: 1630–4
2. Caliezi C, et al. C1-esterase inhibitor: an anti-inflammatory agent and its potential use in the treatment of diseases other than hereditary angioedema. Pharmacol Rev 2000; 52: 91–112
3. de Zwaan C, et al. Continuous 48-h C1-inhibitor treatment, following reperfusion therapy, in patients with acute myocardial infarction. Eur Heart J 2002; 23: 1670–7.

💊 Preparations

Proprietary Preparations

Arg.: Angioneurina†; Berinert P; Austria: Berinert; Cz.: Berinert; Fr.: Esterasine†; Ger.: Berinert; Hung.: Berinert P; Ital.: C1 Inattivatore Umano†; Neth.: Cetor; Switz.: Berinert.
Published May 05, 2019.