Which medications in the drug class C1-Inhibitor Concentrates are used in the treatment of Acquired Angioedema Due to C1 Inhibitor Deficiency?

Updated: May 20, 2020
  • Author: Amanda T Moon, MD; Chief Editor: William D James, MD  more...
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C1-Inhibitor Concentrates

C1-INH is a normal constituent of human blood and is one of the serine proteinase inhibitors (serpins). It regulates activation of the pathway for complement and intrinsic coagulation, and it regulates the fibrinolytic system. Depending on the particular brand, these concentrates are used in the acute treatment of angioedema. They are approved by the US FDA for routine prophylaxis against angioedema attacks and as treatment for acute attacks.

C1 esterase inhibitor, human (Berinert)

This agent is a serine proteinase inhibitor found in human blood that regulates activation of the kinin system, complement pathway, intrinsic coagulation system, and fibrinolytic system. It binds to and neutralizes substrates that activate these systems, thereby suppressing activity. It is available as a pasteurized, lyophilized preparation derived from purified human plasma. One unit corresponds to the mean quantity of C1 inhibitor present in 1 mL of normal fresh plasma. C1 esterase inhibitor is indicated for acute laryngeal, abdominal, and facial angioedema attacks in adolescents and adults with HAE.

C1 inhibitor human (Berinert, Cinryze, Haegarda)

This agent is aerine proteinase inhibitor that increases the plasma concentration of C1 inhibitor activity. Berinert is indicated for the treatment of acute abdominal, facial, or laryngeal attacks of HAE in adults and children. Cinryze and Haegarda are indicated for routine prophylaxis in adults and adolescents. Berinert and Cinryze are administered as an intravenous infusion, whereas Haegarda is administered as a subcutaneous injection.

C1 esterase inhibitor recombinant (Ruconest)

This agent is a human recombinant C1 esterase inhibitor purified from the milk of genetically modified (transgenic) rabbits; it restores the level of functional C1 esterase inhibitor in a patient’s plasma, thereby treating the acute attack of swelling. It is indicated for the treatment of acute HAE attacks in adults and adolescents. It is administered as an intravenous infusion over 5 minutes.

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