Berinert (C1 esterase inhibitor)

Complement 1 (C1)-esterase inhibitor is a naturally occurring inhibitor of certain serine proteases (e.g., C1 complement, kallikrein, coagulation factor XIIa, plasmin) involved in the complement, coagulation (e.g., contact), and fibrinolytic systems. C1-esterase inhibitor (human) is a preparation of C1-esterase inhibitor derived from pooled human plasma.

Complement 1 (C1)-esterase inhibitor is a naturally occurring serine protease inhibitor that principally regulates the activation of the complement and intrinsic coagulation (e.g., contact system) pathways. C1-esterase inhibitor also plays a role in the fibrinolytic system.

Hereditary angioedema (HAE) is an autosomal-dominant genetic disorder characterized by low plasma concentrations of endogenous or functional C1-esterase inhibitor. Such a deficiency leads to uncontrolled activation of the complement and contact system, resulting in increased vascular permeability and angioedema. Replacement therapy with C1-esterase inhibitor (human) increases plasma levels of C1-esterase inhibitor activity and provides an additional source of the enzyme in patients with HAE.

C1-esterase inhibitor regulates the contact system by inhibiting plasma kallikrein and coagulation factor XIIa, thus preventing formation of bradykinin, the presumed mediator of increased vascular permeability in HAE. C1-esterase inhibitor blocks both the spontaneous activation of C1 complement and formation of activated C1 complement, suppressing the classical complement pathway. During these processes, irreversible complexes are formed between the inhibitor and its target protease; the resulting complexes are then inactivated and removed from circulation.

C1-esterase inhibitor also exhibits inhibitory effects on plasmin.

Commercially available Cinryze(R) is a sterile, lyophilized concentrate of highly purified C1-esterase inhibitor (human) derived from pooled human plasma. To reduce the risk of viral transmission, the manufacturing process for Cinryze(R) includes a heat inactivation procedure and additional purification/viral inactivating steps (i.e., polyethylene glycol (PEG) precipitation, nanofiltration).

Berinert(R) is a sterile, lyophilized concentrate of highly purified C1-esterase inhibitor (human) derived from pooled human plasma. The manufacturing process for Berinert(R) includes 3 viral inactivation/reduction steps (i.e., pasteurization, hydrophobic interaction chromatography, nanofiltration) to reduce the risk of viral transmission.

Haegarda(R) is a sterile, lyophilized concentrate of highly purified C1-esterase inhibitor (human) derived from pooled human plasma. The manufacturing process for Haegarda(R) includes 3 viral inactivation/reduction steps (i.e., pasteurization, hydrophobic interaction chromatography, nanofiltration) to reduce the risk of viral transmission.

Following IV administration of Cinryze(R) in clinical studies, plasma concentrations of C1-esterase inhibitor increased immediately (i.e., within 1 hour). Plasma C4 levels appeared to increase 2-24 hours later, indicating consumption of C1-esterase inhibitor and stabilization of the complement activation system. Pharmacokinetic studies conducted with Cinryze(R) demonstrate that in patients with asymptomatic HAE, peak plasma concentrations of C1-esterase inhibitor generally occur approximately 4 hours after a single 1000-unit dose.

The mean half-life of C1-esterase inhibitor following administration of a single 1000-unit dose of Cinryze(R) in such asymptomatic HAE adultpatients was reported to be 56 hours. Using prophylactic doses of 500 or 1000 units given every 3-4 days, the mean steady-state half-life was reported to be approximately 34 hours in pediatric patients 7-11 years of age. Pharmacokinetic data indicate that in pediatric patients 7-11 years of age, total exposure to Cinryze(R) based on AUC and maximum serum concentrations is approximately 30% higher compared to adults at doses of 1000 units given every 3 to 4 days.

Little accumulation is observed when the drug is administered every 3 days. In patients with mild to severe HAE who received single doses (500-1500 units) of Berinert(R), the half-life of the drug was approximately 18 hours in adults and 17 hours in pediatric patients 6-13 years of age. In patients treated with Haegarda(R) 60 IU/kg subcutaneously twice weekly, the median time to peak plasma concentration was approximately 59 hours, and the median half-life was approximately 69 hours.

USES: This medication is used to help prevent swelling problems that occur with a certain immune disease passed down through families (hereditary angioedema-HAE). HAE is caused by low levels or improper function of a natural substance produced by the body (C1-inhibitor). Symptoms such as rapid swelling of the hands, feet, limbs, face, tongue, or throat can occur.

Swelling of the intestinal tract may cause symptoms such as abdominal cramps, diarrhea, or vomiting. Attacks of swelling may occur without reason. However, anxiety, stress, sickness, and surgery may trigger attacks in some people.

This medication increases the level of C1-inhibitor to help prevent attacks of sudden swelling due to hereditary angioedema.

Generic name: C1 esterase inhibitor

The following indications are listed for Berinert:

• Prevention Of Angioedema Attacks In Patient With Hereditary Angioedema

Also known as: Prevention Of Angioedema Attack In Hereditary Angioneurotic Edema, Prevention Of Angioedema Attacks In Patient With Hae, Prophylaxis Against Angioedema Attacks In Patient With Hae.

No ICD codes are available for this drug.