Generic name: complement C1 esterase inhibitor [ KOM-ple-ment-C1 ES-ter-ase-in-HIB-it-or ]
Brand names: Berinert, Cinryze, Haegarda
Dosage forms: intravenous kit (500 intl units), intravenous powder for injection (500 units), subcutaneous powder for injection (2000 intl units; 3000 intl units)
Drug class: Hereditary angioedema agents

Medically reviewed by Drugs.com on Nov 18, 2024. Written by Cerner Multum.

What is complement C1 esterase inhibitor?

Complement C1 esterase inhibitor is a man-made form of a protein in blood that helps control swelling in the body. People with a condition called hereditary angioedema do not have enough of this protein. Hereditary angioedema can cause attacks of swelling and symptoms such as stomach problems or trouble breathing.

Complement C1 esterase inhibitor is used in people with hereditary angioedema. Berinert is used to treat attacks of angioedema. Cinryze and Haegarda are used to prevent attacks of angioedema.

Complement C1 esterase inhibitor may also be used for purposes not listed in this medication guide.

Complement C1 esterase inhibitor side effects

Get emergency medical help if you have signs of an allergic reaction: hives; chest tightness, fast heartbeats, feeling light-headed; wheezing or difficult breathing, blue lips or gums; or swelling of your face, lips, tongue, or throat.

Complement C1 esterase inhibitor may cause serious side effects. Call your doctor at once if you have:

• sudden numbness or weakness on one side of the body;

• pain, swelling, warmth, or redness in an arm or leg;

• sudden severe headache, confusion, problems with vision, speech, or balance;

• chest pain while taking deep breaths; or

• fast heart rate.

Common side effects of complement C1 esterase inhibitor may include:

• unusual or unpleasant taste in your mouth;

• headache, dizziness;

• nausea, vomiting;

• pain, bruising, itching, swelling, bleeding, warmth, or a hard lump where the medicine was injected;

• stuffy nose, sore throat;

• rash; or

• fever.

This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

Related/similar drugs

Warnings

You should not use complement C1 esterase inhibitor if have ever had a life-threatening allergic reaction to complement C1 esterase inhibitor.

Before taking this medicine

You should not use this medicine if have ever had a life-threatening allergic reaction to complement C1 esterase inhibitor.

Tell your doctor if you have ever had:

• a stroke or blood clot;

• heart problems; or

• an "in-dwelling" catheter.

Tell your doctor if you are pregnant or breast-feeding.

Complement C1 esterase inhibitor is made from donated human plasma and may contain viruses or other infectious agents. Donated plasma is tested and treated to reduce the risk of contamination, but there is still a small possibility it could transmit disease. Ask your doctor about any possible risk.

How should I use complement C1 esterase inhibitor?

Follow all directions on your prescription label and read all medication guides or instruction sheets. Use the medicine exactly as directed.

Cinryze or Berinert are given as an infusion into a vein. Haegarda is injected under the skin. A healthcare provider will give your first dose and may teach you how to properly use the medication by yourself.

Read and carefully follow any Instructions for Use provided with your medicine. Prepare an injection only when you are ready to give it. Ask your doctor or pharmacist if you don't understand all instructions.

Seek emergency medical attention after using Berinert to treat an angioedema attack.

Storing unopened vials: Store in a refrigerator or at cool room temperature and use until expiration date. Protect from light and do not freeze.

Each vial (bottle) is for one use only. Throw it away after one use, even if there is still medicine left inside.

After mixing Cinryze, use the injection right away or store at room temperature and use within 3 hours.

After mixing Berinert or Haegarda, use the injection right away or store at room temperature and use within 8 hours.

Complement C1 esterase inhibitor contains no preservative. Once you have pierced the rubber top of a vial with a needle, you must use that vial right away or throw it away.

Use a needle and syringe only once and then place them in a puncture-proof "sharps" container. Follow state or local laws about how to dispose of this container. Keep it out of the reach of children and pets.

Complement C1 esterase inhibitor dosing information

Usual Adult Dose for Hereditary Angioedema:

For routine prophylaxis against angioedema attacks in HAE patients.
Cinryze: 1,000 Units by intravenous infusion at a rate of 1 mL per minute (1,000 units/ 10 mL) every 3 or 4 days.

For treatment of acute abdominal, facial or laryngeal attacks of HAE:
Berinert: 20 units per kg body weight intravenously at a rate of approximately 4 mL per minute (500 units/10 mL).

Usual Pediatric Dose for Hereditary Angioedema:

For treatment of acute abdominal, facial or laryngeal attacks of HAE:
13 years and older:
Berinert: 20 units per kg body weight intravenously at a rate of approximately 4 mL per minute (500 units/10 mL)

What happens if I miss a dose?

Call your doctor for instructions if you miss a dose of Cinryzeor Haegarda.

Keep Cinryze or Haegarda on hand at all times to prevent angioedema, especially while traveling.

What happens if I overdose?

Seek emergency medical attention or call the Poison Help line at 1-800-222-1222.

What should I avoid after using complement C1 esterase inhibitor?

Follow your doctor's instructions about any restrictions on food, beverages, or activity.

What other drugs will affect complement C1 esterase inhibitor?

Tell your doctor about all your other medicines, especially:

• testosterone; or

• birth control pills.

This list is not complete. Other drugs may affect complement C1 esterase inhibitor, including prescription and over-the-counter medicines, vitamins, and herbal products. Not all possible drug interactions are listed here.

C1 esterase inhibitor (human) Biosimilars

Biosimilar and interchangeable products are biological products that are highly similar to and have no clinically meaningful differences from the reference product.

Reference products

These are biological products that have already been approved by the FDA, against which biosimilar products are compared. There is 1 for C1 esterase inhibitor (human).

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Further information

Remember, keep this and all other medicines out of the reach of children, never share your medicines with others, and use this medication only for the indication prescribed.

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

Medical Disclaimer

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