Alpha-1-proteinase inhibitor human

ZEMAIRA is an alpha 1 -proteinase inhibitor (A 1 -PI) indicated for chronic augmentation and maintenance therapy in adults with A 1 -PI deficiency and clinical evidence of emphysema. ZEMAIRA increases antigenic and functional (anti-neutrophil elastase capacity [ANEC]) serum levels and lung epithelial lining fluid (ELF) levels of A 1 -PI. Clinical data demonstrating the long-term effects of chronic augmentation therapy of individuals with ZEMAIRA are not available. The effect of augmentation therapy with ZEMAIRA or any A 1 -PI product on pulmonary exacerbations and on the progression of emphysema in A 1 -PI deficiency has not been demonstrated in randomized, controlled clinical studies. ZEMAIRA is not indicated as therapy for lung disease patients in whom severe A 1 -PI deficiency has not been established.

A 1 -PI deficiency is a chronic, hereditary, autosomal, co-dominant disorder that is usually fatal in its severe form. Low blood levels of A 1 -PI (i.e., below 11 µM) are most commonly associated with progressive, severe emphysema that becomes clinically apparent by the third to fourth decade of life. In addition, PiSZ individuals, whose serum A 1 -PI levels range from approximately 9 to 23 µM, are considered to have a moderately increased risk for developing emphysema, regardless of whether their serum A 1 -PI levels are above or below 11 µM. 2 Not all individuals with severe genetic variants of A 1 -PI deficiency have emphysema. Augmentation therapy with alpha 1 -proteinase inhibitor (human) is indicated only in patients with severe congenital A 1 -PI deficiency who have clinically evident emphysema.

For intravenous use after reconstitution only. The recommended dose of ZEMAIRA is 60 mg/kg body weight administered once weekly. Dose ranging studies using efficacy endpoints have not been performed with ZEMAIRA or any A 1 -PI product. The recommended weekly dose of ZEMAIRA is 60 mg/kg body weight. Administer through a suitable 5 micron infusion filter (not supplied) at room temperature within 3 hours after reconstitution. Do not mix with other medicinal products. Administer through a separate dedicated infusion line. Administer at a rate of approximately 0.08 mL/kg/min as determined by the response and comfort of the patient. Monitor closely the infusion rate and the patient's clinical state, including vital signs, throughout the infusion. Slow or stop the infusion if adverse reactions occur.

ZEMAIRA is contraindicated in patients with a history of anaphylaxis or severe systemic reactions to ZEMAIRA or A 1 -PI protein. ZEMAIRA is contraindicated in immunoglobulin A (IgA)-deficient patients with antibodies against IgA, due to the risk of severe hypersensitivity. History of anaphylaxis or severe systemic reactions to ZEMAIRA or A 1 -PI protein. Immunoglobulin A (IgA)-deficient patients with antibodies against IgA, due to the risk of severe hypersensitivity.

Observe any signs of hypersensitivity such as tachycardia, hypotension, confusion, syncope, oxygen consumption decrease, and pharyngeal edema when administering ZEMAIRA to patients with known hypersensitivity to an A 1 -PI product. Patients with selective or severe IgA deficiency can develop antibodies to IgA and, therefore, have a greater risk of developing potentially severe hypersensitivity and anaphylactic reactions. If anaphylactic or severe anaphylactoid reactions occur, discontinue the infusion immediately. Because ZEMAIRA is made from human blood, it may carry a risk of transmitting infectious agents, e.g., viruses, the variant Creutzfeldt-Jakob disease (vCJD) agent and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent.

Serious adverse reactions reported following administration of ZEMAIRA in pre-licensure clinical trials included one event each in separate subjects of bronchitis and dyspnea, and one event each in a single subject of chest pain, cerebral ischemia and convulsion. The most common adverse reactions (ARs) occurring in at least 5% of subjects receiving ZEMAIRA in all pre-licensure clinical trials were headache, sinusitis, upper respiratory infection, bronchitis, asthenia, cough increased, fever, injection site hemorrhage, rhinitis, sore throat, and vasodilation. Serious adverse reactions identified during postmarketing use were hypersensitivity reactions.

When stored up to 25°C (77°F), ZEMAIRA is stable for the period indicated by the expiration date on its vial label and carton. Avoid freezing, which may damage the diluent vial.