Cinryze

What is Cinryze?

Cinryze (C1 Esterase Inhibitor (Human)) is a C1 esterase inhibitor used for routine prophylaxis against angioedema attacks.

What are the uses of this medicine?

• Cinryze (C1 Esterase Inhibitor (Human)) is an injectable medicine that is used to help prevent swelling and/or painful attacks in children (6 years of age and older), teenagers and adults with Hereditary Angioedema (HAE).
• CINRYZE is manufactured from human plasma purified by a combination of filtration and chromatographic procedures.
• CINRYZE contains C1 esterase inhibitor.

Hereditary Angioedema (HAE):

• HAE is caused by the decreased functioning of a protein called C1 esterase inhibitor, that is present in your blood and helps control inflammation (swelling) and parts of the immune system.

How does this medicine work?

• C1 esterase inhibitor is a normal constituent of human plasma and belongs to the group of serine protease inhibitors (serpins) that includes antithrombin III, alpha1-protease inhibitor, alpha2-antiplasmin, and heparin cofactor II.
• C1 esterase inhibitor, which is usually activated during the inflammatory process, inactivates its substrate by covalently binding to the reactive site.
• C1 esterase inhibitor is the only known inhibitor for the subcomponent of the complement component 1 (C1r), C1s, coagulation factor XIIa, and kallikrein.
• Additionally, C1 esterase inhibitor is the main inhibitor for coagulation factor XIa of the intrinsic coagulation cascade.
• HAE patients have low levels of endogenous or functional C1 esterase inhibitor.
• HAE attacks may be primarily mediated through contact system activation.
• Suppression of contact system activation by C1 esterase inhibitor through the inactivation of plasma kallikrein and factor XIIa is thought to modulate this vascular permeability by preventing the generation of bradykinin.

* Administration of CINRYZE increases plasma levels of C1 inhibitor activity.

Who Should Not Use this medicine ?

This medicine cannot be used in patients who:

• have life-threatening immediate hypersensitivity reactions, including anaphylaxis, to the product.

What drug interactions can this medicine cause?

• No formal drug interaction studies have been conducted with CINRYZE.

Is this medicine FDA approved?

• Initial U.S. Approval: 2008.

How should this medicine be used?

Recommended dosage:

Routine Prophylaxis Dosing against HAE attacks:

• Adults and adolescents (12 years old and above):1,000 Units Intravenous every 3 or 4 days at infusion rate of 1 mL/min (10 min).
• Children (6 to 11 years old): 500 Units Intravenous every 3 or 4 days at infusion rate of 1 mL/min (5 min).

Administration:

• For Intravenous Use Only.
• Prior to reconstitution, protect from light.
• A silicone-free syringe is recommended.
• Store at 36°F to 77°F (2°C to 25°C).
• Do not freeze.
• To obtain a 500 U CINRYZE dose, reconstitute one CINRYZE vial with one vial of Sterile Water for Injection, USP (5 mL).
• To obtain a 1,000 U CINRYZE dose, reconstitute two CINRYZE vials with two vials of Sterile Water for Injection, USP (5 mL each).
• Use aseptic sterile technique.
• Administer at room temperature within 3 hours of reconstitution.

What are the dosage forms and brand names of this medicine?

This medicine is available in fallowing doasage form:

• Approximately 500 Units (lyophilized) in an 8 mL vial.

This medicine is available in fallowing brand namesː

• CINRYZE

What side effects can this medication cause?

The most common side effects of this medicine include:

• headache
• nausea
• rash
• vomiting

• Allergic reactions may occur with CINRYZE.
• Serious blood clots may occur with CINRYZE.

What special precautions should I follow?

Severe hypersensitivity reactions may occur. Have epinephrine immediately available for treatment of acute severe hypersensitivity reaction. Serious arterial and venous thromboembolic (TE) events have been reported at the recommended dose of C1 Esterase Inhibitor (Human) products, including CINRYZE, following administration in patients with HAE. Risk factors may include presence of an indwelling venous catheter/access device, prior history of thrombosis, underlying atherosclerosis, use of oral contraceptives, certain androgens, morbid obesity, and immobility. Benefits of CINRYZE for routine prophylaxis of HAE attacks should be weighed against the risks of TE events in patients with underlying risk factors. Monitor patients with known risk factors for TE events during and after CINRYZE administration. TE events have been reported following administration of a C1 Esterase Inhibitor (Human) product when used off-label at higher than labeled doses. CINRYZE is made from human plasma and may contain infectious agents e.g. viruses and, theoretically, the Creutzfeldt-Jakob disease agent.

What to do in case of emergency/overdose?

Symptoms of overdosage may include:

• There have been no overdosages of CINRYZE reported during clinical studies.

Management of overdosage:

• In the event of an overdose, appropriate supportive treatment should be initiated.

Can this medicine be used in pregnancy?

• There are no data with CINRYZE use in pregnant women to inform a drug associated risk.
• It is unknown whether CINRYZE can cause fetal harm when administered to a pregnant woman or can affect reproduction capacity.
• CINRYZE should be given to a pregnant woman only if clearly needed.

Can this medicine be used in children?

• The safety and effectiveness of CINRYZE have been evaluated in 12 pediatric subjects with HAE (age range 7 to 11 years old).

What are the active and inactive ingredients in this medicine?

Active ingredient:

• HUMAN C1-ESTERASE INHIBITOR

Inactive ingredients:

• SODIUM CHLORIDE
• SUCROSE
• TRISODIUM CITRATE DIHYDRATE
• VALINE
• ALANINE
• THREONINE

Who manufactures and distributes this medicine?

Manufactured by:

• ViroPharma Biologics LLC
• Lexington, MA

• CINRYZE® is a trademark of ViroPharma Biologics LLC, a Takeda Company.

What should I know about storage and disposal of this medication?

• Do not freeze CINRYZE.
• Store CINRYZE in a refrigerator or at room temperature between 36°F to 77°F (2°C to 25°C).
• Keep CINRYZE in the original carton to protect it from light.
• Do not use CINRYZE after the expiration date on the vial.

• Dailymed label info on Cinryze
• FDA Cinryze

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