CINRYZE

CINRYZE is a prescription medication given by injection to help prevent swelling and painful attacks in individuals aged 6 and older, including children, teenagers, and adults.

The following medications are used for the treatment of hereditary angioedema:

1. Ruconest® (C1 esterase inhibitor, recombinant)
2. Berinert®, Cinryze®, Haegarda® (C1 inhibitor, human)
3. Kalbitor® (Ecallantide)
4. Firazyr® (Icatibant)
5. Takhzyro® (Lanadelumab)
6. Orladeyo® (Berotralstat)

If the airway is blocked due to swelling of the tongue or throat, angioedema can become life-threatening.

To effectively manage angioedema attacks, it is important to discontinue the causative drug and ensure proper airway management. Additionally, since the majority of angioedema cases are histamine-mediated, initial treatment should focus on addressing this aspect. This involves administering antihistamines, glucocorticoids, and epinephrine.

Background: Hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) is a condition characterized by recurring swelling in the subcutaneous or submucosal tissues. The symptoms typically start between the ages of 5 and 11 and tend to worsen during puberty. However, it is important to note that attacks can happen at any age and continue to recur throughout a person’s life.

Antihistamines that do not cause drowsiness are the recommended treatment for hives and angioedema. These medications effectively alleviate itching, swelling, and other symptoms associated with allergies. Both over-the-counter and prescription options are available.

Treatment for angioedema typically involves the use of medication. While many cases of angioedema improve on their own within a few days, antihistamines and oral steroids are commonly prescribed to alleviate swelling in cases of allergic and idiopathic angioedema.

There are various medications available to prevent or alleviate attacks. For mild cases, over-the-counter antihistamines like Benadryl, Zyrtec, Allegra, or Claritin can be used. It’s important to note that Benadryl may cause drowsiness. Your doctor may also prescribe antihistamines for you.

The typical treatment approach includes a combination of prophylaxis, the use of androgens or antifibrinolytic drugs, and the administration of C’1 esterase inhibitor concentrate for acute attacks and prior to surgery or other invasive procedures.

The inappropriate activation of the contact-kinin system, release of bradykinin, increased vascular permeability, and angioedema are caused by a deficiency of C1-INH. AAE-C1-INH is typically diagnosed after the age of 40.

To ensure the needle stays in place, secure it with tape. Then, remove the tourniquet. Double-check that the needle is inserted into a vein. Administer the dissolved CINRYZE (C1 esterase inhibitor [human]) slowly over a period of 5 minutes for a 500 IU dose or 10 minutes for a 1,000 IU dose, at a rate of approximately 1 mL per minute.

CINRYZE can be self-administered by the patient or administered by a caregiver or healthcare provider for the prevention of HAE attacks. It is recommended to discuss with the patient and/or their caregiver to determine the most suitable administration option for them.

Outpatient treatment alone is typically sufficient for managing the majority of angioedema cases. First-line treatment often involves the use of second-generation antihistamines such as cetirizine, desloratadine, fexofenadine, levocetirizine, and loratadine.

To ensure proper administration, follow these guidelines for CINRYZE:

– Administer CINRYZE by intravenous injection at a rate of 1 mL per minute.
– For a single dose of 500 IU (reconstituted in 5 mL) of CINRYZE, administer it over 5 minutes.
– For a single dose of 1,000 IU (reconstituted in 10 mL) of CINRYZE, administer it over 10 minutes.

In the majority of cases, symptoms develop rapidly and can be effectively treated with antihistamines, epinephrine, or steroids. However, in certain instances, the angioedema may not respond to these standard treatments and requires more intensive therapy, such as omalizumab or cyclosporine.

If the patient is diagnosed with stage I or II angioedema, it is recommended to observe them for several hours to assess if the condition progresses. If there is further progression, the patient should be admitted for further care. However, if the patient is at stage I, they can be discharged after several hours of monitoring. On the other hand, patients at stage II should be observed for a longer period of time (1-8,62).

The administration of steroids should be done as soon as possible, not for immediate benefits, but to prevent the production of leukotrienes and late-phase reactions, which are rare but can be prevented. The recommended dose of steroids for adults is currently 200 mg of hydrocortisone administered intravenously.

Epinephrine intramuscularly, antihistaminergic medications, and steroids are the recommended treatments for histamine-mediated angioedema. However, these medications are not effective for bradykinin-mediated forms. For bradykinin-mediated angioedema, other medications such as C1-INH protein replacement, kallikrein inhibitor, and bradykinin receptor antagonists should be considered.

Berinert is used to treat sudden attacks of HAE in adults and children that affect the abdomen, face, or throat. Cinryze and Haegarda are used for regular prevention in adults and teenagers. Berinert and Cinryze are given through an intravenous infusion, while Haegarda is given as a subcutaneous injection.

In cases of angioedema, there is a risk of life-threatening airway blockage due to throat swelling and anaphylactic reactions. Treatment may involve the use of histamine blockers (H1 and H2), steroids, and, in severe cases, epinephrine administered through injection into the muscle or under the skin.

The primary approach to managing ACEi-induced angioedema involves discontinuing the use of ACEi medication and strictly avoiding any other drugs from the same group. Although episodes of swelling may continue to occur for a period of three months or longer after stopping ACEi, they typically become less frequent and severe over time.

Cinryze is a C1 inhibitor that is made from human plasma. C1 inhibitor is a natural part of human blood and belongs to a group of proteins called serine proteinase inhibitors (serpins). The main role of C1 inhibitor is to control the activation of the complement and intrinsic coagulation (contact system) pathway.

Berinert is a medication that contains a concentrated form of C1 esterase inhibitor (C1-INH). It is derived from healthy blood donors and comes in the form of a single-use powder. During an acute hereditary angioedema (HAE) attack, Berinert is dissolved and injected into the bloodstream to increase the levels of functional C1-INH.

Ruconest is obtained by purifying it from the milk of transgenic rabbits, while Cinryze is a stable and sterile preparation of C1 esterase inhibitor derived from human plasma. The activation of the contact system may play a significant role in the increased vascular permeability and clinical symptoms observed during HAE attacks.

Package insert / product label for c1 esterase inhibitor recombinant injection, powder for solution, used as a hereditary angioedema agent.

The inappropriate activation of the contact-kinin system, release of bradykinin, increased vascular permeability, and angioedema are caused by a deficiency of C1-INH. AAE-C1-INH is typically diagnosed after the age of 40.

Firazyr, the third-ranked drug by Shire, is priced at $32,468 per month. It is included in the top 10 list of the most expensive retail drugs in the U.S., according to NowPatient Rx Advantage Card. Firazyr received approval in 2011 and is used to treat patients with hereditary angioedema following an attack.

You can administer additional doses of FIRAZYR if your symptoms are not resolved or if they occur again after your first dose. These additional doses should be given at least 6 hours apart, and you should not exceed a total of 3 doses within a 24-hour period.

FIRAZYR is approved for treating sudden episodes of hereditary angioedema (HAE) in adults who are 18 years of age and older.

Hereditary angioedema (HAE) is a genetic disease that is passed down through families and is caused by a deficiency or dysfunction of the C1-inhibitor protein. This condition leads to swelling in the upper airway, skin, and/or gastrointestinal tract.

Berotralstat is prescribed to prevent severe swelling attacks caused by hereditary angioedema (HAE), an inherited immune disease. It functions by binding to kallikrein, a natural substance produced by the body, and inhibiting its impact.

Cinryze, manufactured by Shire, is priced at $44,141 for a standard one-month supply of 16 vials. This medication is specifically designed to treat hereditary angioedema, a rare and potentially life-threatening genetic disorder that results in swelling in different areas of the body such as the hands, face, and throat.

The cost of Cinryze is approximately INR 3.9 lakh per vial.

Cinryze (c1 esterase inhibitor [human]) Freeze Dried Powder is a synthetic version of a naturally occurring protein in the blood that aids in regulating inflammation in the body. It is prescribed for individuals with hereditary angioedema. The medication is also available under the brand name Berinert and is used to manage angioedema attacks.

The survey also evaluated the total annual cost of having and treating HAE in the USA, which was found to be $44,597 per patient on average.

The active ingredient in Cinryze is human C1 inhibitor, a protein derived from human blood. This protein is essential for regulating the ‘complement’ and ‘contact’ systems, which are groups of proteins in the blood that combat infections and trigger inflammation.

The medications utilized for angioedema treatment encompass antihistamines, corticosteroids (anti-inflammatory medicines), and epinephrine shots (which individuals with a history of severe symptoms can carry for emergency use).

Icatibant is the generic form of Firazyr. Generics are identical to brand-name drugs in terms of safety and effectiveness. Additionally, generics are typically more affordable than their brand-name counterparts.

It is believed that HAE affects only 1 in 50,000 individuals worldwide. Additionally, it is estimated that there are approximately 6,000 people in the United States who have HAE.

Aurobindo Pharma has announced that the U.S. Food and Drug Administration (FDA) has given approval for a generic version of Firazyr (icatibant injection) to treat hereditary angioedema (HAE). This generic medication is intended for adults aged 18 and above and is used to treat acute swelling attacks of HAE, just like the brand-name medication.

FIRAZYR is indicated for the treatment of acute episodes of hereditary angioedema (HAE) in adults who are 18 years of age or older.

The price of a 3 milliliter supply of icatibant subcutaneous solution (10 mg/mL) can vary, but it is typically around $5,194, depending on the pharmacy you choose.

Firazyr is given as a subcutaneous injection to alleviate symptoms of angioedema, such as skin or stomach swelling and pain. The patient should experience relief within approximately 2 hours after the injection, although it may take up to 8 hours for them to fully recover.