Cinryze: 7 things you should know

Medically reviewed by Carmen Pope, BPharm. Last updated on May 15, 2024.

1. How it works

• Cinryze is a brand (trade) name for a man-made form of a protein called C1 esterase inhibitor, that naturally occurs in our blood. Cinryze may be used to prevent attacks of hereditary angioedema (HAE) in adults and children aged 6 years and older. HAE is a rare genetic condition that occurs in people with low levels of certain proteins (C1-INH and C4) in their bodies.
• The main purpose of the C1 inhibitor protein is to regulate the coagulation pathway (the process that causes our blood to clot), the fibrinolytic system (the process that removes and degrades clots after blood vessels are repaired), and to control the production of bradykinin, which is a protein fragment that increases the movement of fluid through blood vessel walls, resulting in swelling and inflammation.
• Cinryze increases levels of C1 protein, which reduces bradykinin production, decreasing the permeability of blood vessels and reducing swelling. Cinryze works by replacing missing C1 protein in the blood of people with HAE, preventing the generation of bradykinin which is a protein fragment responsible for increasing the permeability of blood vessels.
• Cinryze belongs to the class of medicines known as C1 esterase inhibitors. It may also be called a hereditary angioedema agent.

2. Upsides

• May be used to prevent angioedema attacks in adults, adolescents, and children aged 6 years and older with hereditary angioedema (HAE).
• Reduces swelling in the limbs, face, intestinal tract, and airway.
• The usual dosage is 1000 units intravenously every 3 or 4 days in adults and adolescents aged 12 years and older, up to a maximum of 2500 units (not exceeding 100 units/kg) if the response is inadequate. Children aged 6 to 11 years should receive 500 units IV every 3 to 4 days, up to a maximum of 1000 units every 3 or 4 days.
• Infused at a rate of 1mL/min; the average infusion time is 10 minutes in adults and 5 minutes in children.

3. Downsides

If you are between the ages of 18 and 60, take no other medication or have no other medical conditions, side effects you are more likely to experience include:

• Headache, nausea, rash, itch, vomiting, and fever are the most common side effects reported.
• Catheter site pain and redness around the injection site have also been reported.
• Severe hypersensitivity reactions (severe allergies) have been reported. Advise people to report any signs of an allergic reaction such as hives, rash, fast heartbeat, difficulty breathing (wheezing), chest tightness, feeling light-headed or faint, swelling of your face, lips, tongue, or throat during or after the administration of Cinryze and to seek immediate help. Note that hypersensitivity reactions may have similar symptoms to HAE attacks. If a hypersensitivity reaction occurs, discontinue Cinryze and administer appropriate treatment, including epinephrine if necessary.
• Serious thromboembolic events (blood clots) have been reported with C1 esterase inhibitor products, such as Cinryze, The risk is greater in those with an indwelling catheter, prior history of thrombosis, underlying atherosclerosis, use of oral contraceptives, certain androgens, morbid obesity, or immobility. Weigh up the benefits of Cinryze in these people versus the risk of an event.
• Because Cinryze is made from human blood there is a risk of transmitting infectious agents, such as viruses, and theoretically, the Creutzfeldt-Jakob disease vector.
• Needs to be given by slow injection every three or four days to prevent angioedema attacks in people with HAE.
• Not suitable for people who have developed life-threatening, immediate hypersensitivity reactions, including anaphylaxis, to Cinryze.
• There is also the potential for immunogenicity and antibody formation.
• Usually administered by a healthcare professional, although some people can be taught how to self-administer it. There are alternative C1 esterase inhibitors (such as Haegarda) that are easier to infuse at home or in other non-healthcare settings to prevent angioedema attacks.
• Does not treat attacks of HAE. Other medications, such as Berinert or Ruconest do this.
• The safety of Cinryze in children under the age of 6 has not been established.
• There is no data regarding the use of Cinryze in pregnant women, and animal studies have not been conducted. It is unknown if Cinryze can cause fetal harm or affect reproduction capacity. Only give to pregnant women if needed. There is no data regarding the presence of Cinryze in human milk or its effects on milk production.
• No generic is available

Note: In general, seniors or children, people with certain medical conditions (such as liver or kidney problems, heart disease, diabetes, seizures) or people who take other medications are more at risk of developing a wider range of side effects. View complete list of side effects

4. Bottom Line

• Cinryze works by replacing missing C1 protein in the blood of adults and children over the age of 6 with hereditary angioedema (HAE) preventing the generation of bradykinin and angioedema attacks. Usually administered by a healthcare professional every 3 or 4 days, although some people can be taught how to self-administer. A headache, nausea, rash, itch, vomiting, or fever are the main side effects reported.

5. Tips

• If you have any signs of an allergic reaction during your Cinryze infusion or after, immediately tell your healthcare professional. Signs of an allergic reaction may include hives, rash, fast heartbeat, difficulty breathing (wheezing), chest tightness, feeling light-headed or faint, and swelling of your face, lips, tongue, or throat.
• Also report any signs of a blood clot, such as swelling, pain, discoloration, a feeling or warmth, in an arm or leg, shortness of breath, chest pain or discomfort that worsens on deep breathing, numbness or weakness on one side of the body.
• Because Cinryze is a blood product, it does carry a risk for transmission of infectious agents such as viruses, but this is unlikely because the risk of transmitting disease has been reduced by carefully selecting blood donors, testing donors for infections, and inactivating viruses during the manufacturing process.
• Cinryze is usually administered every three or four days. Keep to your regular dosing schedule and do not miss any doses. If you do miss a dose, administer it as soon as you remember, but do not double up on doses. If you are traveling, take enough Cinryze with you to continue with your regimen. Do not attempt to self-administer unless you have been taught how to do so by your healthcare provider. Make sure you follow their instructions about administration. Cinryze needs to be injected into a superficial vein, and not into surrounding tissues or an artery.
• Cinryze has not been studied in pregnant or breastfeeding women. Talk to your healthcare provider about the risk of taking this drug during pregnancy or breastfeeding especially if you are considering trying to become pregnant. Tell your doctor if you inadvertently become pregnant.

6. Response and effectiveness

• Research has shown that the administration of Cinryze significantly reduces HAE attacks. A study that involved 24 people demonstrated that Cinryze reduced HAE attacks by 100% in 4 of them, and by more than 50% in 11 people. 2 people experienced more attacks while being administered Cinryze than without it.
• The average number of attacks in people prescribed Cinryze was 6.1 compared with 12.7 in those taking a placebo (an inactive treatment). People treated with Cinryze had a 66% reduction in the days of swelling and decreases in the severity and duration of attacks.

7. Interactions

Medicines that interact with Cinryze may either decrease its effect, affect how long it works, increase side effects, or have less of an effect when taken with Cinryze. An interaction between two medications does not always mean that you must stop taking one of the medications; however, sometimes it does. Speak to your doctor about how drug interactions should be managed.

Common medications that may interact with Cinryze include:

• anti-cancer medications such as carfilzomib
• danazol
• epoetins
• hormones, such as estrogen, estradiol, nandrolone, or testosterone
• lenalidomide
• raloxifene
• streptokinase
• thalidomide
• thrombolytics such as alteplase, anistreplase, or urokinase.

Note that this list is not all-inclusive and includes only common medications that may interact with Cinryze. You should refer to the prescribing information for Cinryze for a complete list of interactions.

References

• Cinryze (c1 esterase inhibitor [human]). Updated 02/2023. Takeda Pharmaceuticals Inc. https://www.drugs.com/pro/cinryze.html

Further information

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

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

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