Alpha-1 proteinase inhibitor

U.S. BRAND NAMES — Aralast; Prolastin®; Zemaira®
PHARMACOLOGIC CATEGORY Antitrypsin Deficiency Agent
DOSING: ADULTS — Alpha1-antitrypsin deficiency: I.V.: 60 mg/kg once weekly
DOSING: ELDERLY — Refer to adult dosing.
DOSAGE FORMS — Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Injection, powder for reconstitution [preservative free]:
Aralast, Prolastin®: 500 mg, 1000 mg [packaged with diluent]
Zemaira®: 1000 mg [packaged with diluent]
DOSAGE FORMS: CONCISE Injection, powder for reconstitution[preservative free]: Aralast, Prolastin®: 500 mg, 1000 mg Zemaira®: 1000 mg
GENERIC EQUIVALENT AVAILABLE — No
ADMINISTRATION — For I.V. infusion only; reduce rate or temporarily discontinue infusion if adverse effects occur. Infuse at ~0.08 mL/kg/minute; actual rate may be increased or decreased based on patient response. Emergency equipment (including epinephrine) should be available during infusion. Do not mix with other agents or solutions.
USE — Replacement therapy in congenital alpha1-antitrypsin deficiency with clinical emphysema
ADVERSE REACTIONS SIGNIFICANT >10%: Hepatic: ALT/AST increased (11%; ~4 times ULN)
1% to 10%: Respiratory: Pharyngitis (2%)
<1% (Limited to important or life-threatening): Allergic reactions, bilateral pulmonary infiltrates, bronchitis, chest pain, chills, cough increased, dizziness, dyspnea, fever <102ºF (delayed up to 12 hours after treatment), flu-like syndrome, headache, hypotension, leukocytosis, lightheadedness, pain, pruritus, rash, sinusitis, somnolence, tachycardia, vasodilation, vision abnormal. Severe reactions (anaphylaxis) may occur in patients with anti-IgA antibody.
CONTRAINDICATIONS — Hypersensitivity to any component of the formulation or other A1-PI products; selective IgA deficiency with known anti-IgA antibody
WARNINGS / PRECAUTIONS — Use caution in patients at risk for fluid overload. Products are made from pooled human plasma; risk of infection is theoretical, however, any infections thought to be transmitted by these products should be reported to the manufacturer. Hepatitis B immunization may be considered. Safety and efficacy in pediatric patients have not been established.
PREGNANCY RISK FACTOR — C (show table)
PREGNANCY IMPLICATIONS — Reproduction studies have not been conducted.
LACTATION — Excretion in breast milk unknown/use caution
DIETARY CONSIDERATIONS — Sodium content of 1 L after reconstitution:
Aralast™: 230 mEq/L
Prolastin®: 100-210 mEq/L
MONITORING PARAMETERS — Alpha1-PI serum levels; vital signs during infusion
CANADIAN BRAND NAMES — Prolastin®
INTERNATIONAL BRAND NAMES — Prolastin (CA)
MECHANISM OF ACTION — Alpha1-antitrypsin (AAT) is the principle protease inhibitor in serum. Its major physiologic role is to render proteolytic enzymes (secreted during inflammation) inactive. A decrease in AAT, as seen in congenital AAT deficiency, leads to increased elastic damage in the lung, causing emphysema.
PHARMACODYNAMICS / KINETICS Half-life elimination: Metabolic: 5.9 days (Aralast™)
Time to peak, serum: Threshold levels achieved after 3 weeks
PATIENT INFORMATION — This medication is given by injection only.