About CINRYZE® (C1 Esterase Inhibitor [Human])
CINRYZE (C1 Esterase Inhibitor [human]) is indicated for routine prophylaxis against attacks of Hereditary Angioedema (HAE), also known as C1 esterase inhibitor deficiency, a disease characterized by unpredictable swelling, which can be dangerous and disfiguring and can cause life-threatening emergencies. CINRYZE is a sterile, stable, lyophilized preparation of C1 esterase inhibitor derived and manufactured from human plasma, and purified by a combination of nanofiltration and chromatographic procedures.
CINRYZE Mechanism of Action
C1 esterase inhibitor is a normal constituent of human blood and is one of the serine proteinase inhibitors (serpins). The primary function of C1 esterase inhibitor is to regulate the activation of the complement and intrinsic coagulation (contact system) pathway. C1 esterase inhibitor also regulates the fibrinolytic system. Regulation of these systems is performed through the formation of complexes between the proteinase and the inhibitor, resulting in inactivation of both and consumption of the C1 esterase inhibitor.
HAE patients have low levels of endogenous or functional C1 esterase inhibitor. Although the events that induce attacks of angioedema in HAE patients are not well defined, it is thought by some that increased vascular permeability and the clinical manifestation of HAE attacks are 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 bradykinin6. Administration of CINRYZE increases plasma levels of C1 esterase inhibitor activity.
How CINRYZE is Administered
CINRYZE (C1 Esterase Inhibitor [human]) therapy is administered as an intravenous injection rate of 1 mL per minute over 10 minutes at a dose of 1000 units for routine prophylaxis against HAE attacks. One unit (U) of CINRYZE corresponds to the mean quantity of C1 esterase inhibitor present in 1 mL of normal fresh plasma. CINRYZE is a lyophilized preparation available in a single-use vial that contains 500 Units (U) of human C1 esterase inhibitor. Each vial must be reconstituted with 5 mL Sterile Water for Injection, USP (diluent). Two reconstituted vials must be used to make a single, 1000 Unit, dose.
Indication
CINRYZE (C1 Esterase Inhibitor [human]) is indicated for routine prophylaxis against angioedema attacks in adolescent and adult patients with Hereditary Angioedema (HAE).
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Contraindications and Warnings
Severe hypersensitivity reactions may occur. Because hypersensitivity reactions and HAE attacks may have similar symptoms, distinguishing between the two is important. Epinephrine should be immediately available to treat any acute severe hypersensitivity reaction and Cinryze should be discontinued.
Thrombotic events have occurred in patients receiving CINRYZE for routine prophylaxis, and in patients receiving off-label high dose C1 inhibitor therapy. Monitor patients with known risk factors for thrombotic events.
CINRYZE is made from human plasma. Products made from human plasma may contain infectious agents (e.g. viruses and, theoretically, the Creutzfeldt-Jakob Disease agent). The risk that such products will transmit an infectious agent has been reduced by screening donors, testing for the presence of infections, and by inactivating or removing a broad range of viruses during the manufacturing process.
In the clinical trial, the most common adverse reactions observed by ≥ 5% of the 24 evaluable subjects after receiving CINRYZE were upper respiratory tract infection, sinusitis, rash, and headache.
Before prescribing CINRYZE, please read the Full Prescribing Information, which can be accessed here.