C1 esterase inhibitor recombinant

Action and Spectrum

Actions and spectrum: 

C1 esterase inhibitor recombinant, also known as conestat alfa used for the treatment of hereditary angioedema (HAE) attacks. It is a recombinant form of the C1 esterase inhibitor protein, which helps regulate the complement system and bradykinin release.

By restoring the deficient or dysfunctional C1 esterase inhibitor, helps prevent the excessive swelling and inflammation characteristic of HAE attacks. Its spectrum of action specifically targets the underlying pathophysiology of HAE, providing relief from symptoms and reducing the severity and duration of attacks. 

Drug Interaction

Adverse Reaction

Frequency defined 

1-10% 

Diarrhea (2%) 

Nausea (2%) 

Headache (9%)  

Post marketing Reports 

Rash 

Abdominal pain 

Black Box Warning

Black Box Warning: 

There are no black box warnings specifically associated with C1 esterase inhibitor recombinant. 

Contraindication / Caution

Contraindication/Caution: 

Contraindication: 

C1 esterase inhibitor recombinant is contraindicated in individuals with a known hypersensitivity to conestat alfa or any of its components. Additionally, there may be specific contraindications based on the formulation and specific indications of conestat alfa. It is important to refer to the prescribing information, package inserts, or consult with a healthcare professional for the complete and most up-to-date list of contraindications associated with C1 esterase inhibitor recombinant.  

Caution: 

• Hypersensitivity reactions: Monitor patients for signs of hypersensitivity, including rash, itching, swelling, or difficulty breathing. Discontinue treatment and initiate appropriate medical management if a severe allergic reaction occurs. 
• Transmissible infectious agents: C1 esterase inhibitor recombinant is derived from human plasma. The potential for transmission of infectious agents cannot be eliminated. 
• Pregnancy and lactation: Use during pregnancy or breastfeeding should be carefully considered of the potential risks and benefits. Consult with a healthcare professional for individualized guidance. 
• Pediatric use: Safety & efficacy in pediatric patients have not been established. Use in this population should be done with caution and based on careful evaluation.

Comorbidities: 

• Cardiovascular disease: Patients with a history of cardiovascular disease like myocardial infarction, heart failure, or arrhythmias, may require close monitoring during treatment with C1 esterase inhibitor recombinant. 
• Renal impairment: C1 esterase inhibitor recombinant is primarily cleared renally. Patients with renal impairment may require dosage adjustments or more frequent monitoring to ensure appropriate drug levels and efficacy. 
• Hepatic impairment: C1 esterase inhibitor recombinant is metabolized in the liver. Patients with hepatic impairment may require dosage adjustments or more frequent monitoring to ensure appropriate drug levels and efficacy. 
• Immunocompromised state: Individuals with compromised immune function, such as those receiving immunosuppressive therapy or with underlying immunodeficiency conditions, may have an increased risk of infections. Careful monitoring for signs of infection is recommended in these patients. 

Pregnancy / Lactation

Pregnancy consideration: pregnancy category: C 

Lactation: N/A  

Pregnancy category: 

• Category A: well-controlled and Satisfactory studies show no risk to the fetus in the first or later trimester. 
• Category B: there was no evidence of risk to the fetus in animal studies, and there were not enough studies on pregnant women. 
• Category C: there was evidence of risk of adverse effects in animal reproduction studies, and no adequate evidence in human studies must take care of potential risks in pregnant women.   
• Category D: adequate data with sufficient evidence of human fetal risk from various platforms, but despite the potential risk, and used only in emergency cases for potential benefits. 
• Category X: Drugs listed in this category outweigh the risks over benefits. Hence these categories of drugs need to be avoided by pregnant women. 
• Category N: There is no data available for the drug under this category. 

Pharmacology

Pharmacology: 

C1 esterase inhibitor recombinant binds to and inhibits the enzymatic activity of C1 esterase, which is responsible for cleaving complement proteins and activating the contact system. By inhibiting C1 esterase, the medication helps regulate the production of bradykinin, thereby reducing the symptoms of angioedema. 

C1 esterase inhibitor recombinant modulates the activity of the complement system, specifically the classical pathway. It prevents the excessive activation of the complement cascade, which can lead to tissue inflammation and edema.  

Pharmacodynamics: 

The pharmacodynamics of C1 esterase inhibitor recombinant involves its ability to restore or increase the levels of functional C1 esterase inhibitor (C1-INH) in the body. C1-INH plays a crucial role in regulating the activity of complement system proteins and other proteases involved in inflammation.

By replenishing or augmenting C1-INH levels, C1 esterase inhibitor recombinant helps to inhibit excessive activation of the complement system and prevent uncontrolled inflammatory responses. This can be beneficial in conditions associated with C1-INH deficiency, such as hereditary angioedema (HAE).  

Pharmacokinetics: 

Absorption 

C1 esterase inhibitor recombinant is administered via intravenous (IV) infusion. Upon administration, the medication is rapidly distributed into the bloodstream. 

Distribution 

C1 esterase inhibitor recombinant is distributed throughout the body, including extravascular tissues. It primarily targets sites of inflammation and tissue damage. 

Metabolism 

C1 esterase inhibitor recombinant is a protein-based medication and undergoes degradation by proteolytic enzymes in the body. The exact metabolic pathways and specific enzymes involved in its degradation have not been fully elucidated. 

Elimination and excretion 

The excretion route of C1 esterase inhibitor recombinant is not well-defined. However, as a protein, it is expected to undergo renal clearance and elimination from the body. 

It is important to note that specific details about the pharmacokinetics and elimination half-life of C1 esterase inhibitor recombinant may vary based on individual patient characteristics and the specific dosing regimen used. 

Adminstartion

Administration: 

C1 esterase inhibitor recombinant is administered through intravenous (IV) infusion. The exact administration details, including dosage and infusion rate, should be determined by a healthcare professional based on the individual patient’s condition and the specific indication for treatment. 

The medication is typically administered in a healthcare setting such as a hospital or clinic, where healthcare professionals can monitor the patient closely during the infusion. The infusion is usually given over a specific time period, and the healthcare professional will ensure that the medication is administered properly and safely. 

Patient Information Leaflet

Patient information leaflet 

Generic Name: C1 esterase inhibitor recombinant 

Pronounced: (C-one est-er-ays in-HIB-i-ter ree-KOM-bi-nant)  

Why do we use C1 esterase inhibitor recombinant? 

• Acute Hereditary Angioedema (HAE) Attacks: It is used in the treatment of acute hereditary angioedema (HAE) in adult and adolescent patients. HAE is a genetic disorder characterized by swelling of face, extremities, gastrointestinal tract, and airway. 
• Prophylaxis of Hereditary Angioedema (HAE): It may be used for long-term prophylactic treatment in patients with frequent and severe HAE attacks to reduce the frequency, severity, and duration of attacks.