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Alpha1-Proteinase Inhibitor

Pronunciation

(al fa won PRO tee in ase in HI bi tor)

Index Terms

  • A1 - PI
  • Alpha1 - Antiprotease
  • Alpha1 - Antitrypsin
  • Alpha1 - PI
  • Alpha1 - Proteinase Inhibitor, Human
  • α1-PI

Dosage Forms

Excipient information presented when available (limited, particularly for generics); consult specific product labeling. [DSC] = Discontinued product

Solution, Intravenous [preservative free]:

Glassia: 1000 mg/50 mL (1 ea) [latex free]

Solution Reconstituted, Intravenous:

Prolastin: 500 mg (1 ea [DSC])

Zemaira: 1000 mg (1 ea)

Solution Reconstituted, Intravenous [preservative free]:

Aralast NP: 400 mg (1 ea [DSC]); 500 mg (1 ea); 800 mg (1 ea [DSC]); 1000 mg (1 ea) [contains polyethylene glycol, polysorbate 80]

Prolastin-C: 1000 mg (1 ea)

Suspension Reconstituted, Intravenous:

Prolastin: 1000 mg (1 ea [DSC])

Brand Names: U.S.

  • Aralast NP
  • Glassia
  • Prolastin [DSC]
  • Prolastin-C
  • Zemaira

Pharmacologic Category

  • Antitrypsin Deficiency Agent
  • Blood Product Derivative

Pharmacology

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.

Distribution

Vd: ~3.5 L

Time to Peak

Serum: ~1 hour; threshold levels achieved after 3 weeks

Half-Life Elimination

Metabolic: ~5-6 days

Use: Labeled Indications

Alpha1-proteinase inhibitor deficiency: Long-term augmentation and maintenance therapy in adults with hereditary deficiency of alpha1-antitrypsin (AAT) with clinically evident emphysema.

Limitations of use:

Clinical data demonstrating the long-term effects of chronic augmentation or maintenance therapy of individuals with alpha1-proteinase inhibitor are not available.

The effect of augmentation therapy with any alpha1-proteinase inhibitor on pulmonary exacerbations and on the progression of emphysema in AAT deficiency has not been demonstrated in randomized, controlled clinical trials.

Alpha1-proteinase inhibitors are not indicated as therapy for patients with lung disease in whom hereditary AAT deficiency has not been established.

Contraindications

Hypersensitivity to any component of the formulation or other A1-proteinase inhibitor products; IgA deficient patients with known anti-IgA antibody.

Dosing: Adult

Alpha1-antitrypsin deficiency: IV: 60 mg/kg once weekly

Dosing: Geriatric

Refer to adult dosing.

Dosing: Renal Impairment

There are no dosage adjustments provided in the manufacturer’s labeling.

Dosing: Hepatic Impairment

There are no dosage adjustments provided in the manufacturer’s labeling.

Reconstitution

Reconstitute lyophilized powder with provided diluent (SWFI). Allow product and diluent to reach room temperature (if refrigerated) prior to reconstitution. Filtering requirements during preparation vary by product; consult manufacturer recommendations. May pool vial contents by transferring into a sterile container for IV infusion. To mix, swirl; do not shake. Use within 3 hours of reconstitution.

Aralast NP: Each 500 mg and 1000 mg vial should be diluted with 25 mL and 50 mL of diluent, respectively

Prolastin-C: Each 1000 mg vial should be diluted with 20 mL of diluent

Zemaira: Each 1000 mg vial should be diluted with 20 mL of diluent

Administration

For IV infusion only. Administer within 3 hours of preparation or within 3 hours of entering the vial (Glassia); products do not contain a preservative. Do not mix with other agents or solutions. If adverse reactions occur during administration, rate may be decreased or temporarily interrupted. Some products require an in-line filter for administration; consult specific product labeling.

Aralast NP, Glassia: Infuse at rate of ≤0.2 mL/kg/minute. Glassia may be self-administered by the patient/caregiver at home after appropriate training

Prolastin-C, Zemaira: Infuse at ~0.08 mL/kg/minute (rate may be increased or decreased based on patient comfort).

Dietary Considerations

Some products may contain sodium.

Storage

Aralast NP: Prior to reconstitution, store intact vials up to 25°C (77°F); do not freeze. Keep vial in carton to protect from light. Use within 3 hours of reconstitution.

Glassia: Prior to use, store intact vials between 2°C and 8°C (36°F and 46°F); do not freeze. May also be stored up to 25°C (77°F) for up to one month. Keep vial in carton until ready for use. Use within 3 hours of entering the vial.

Prolastin-C: Prior to reconstitution, store intact vials up to 25°C (77°F); avoid freezing. Use within 3 hours of reconstitution.

Zemaira: Prior to reconstitution, store intact vials up to 25°C (77°F); avoid freezing. Use within 3 hours of reconstitution.

Drug Interactions

There are no known significant interactions.

Adverse Reactions

Actual incidence may vary by product.

Cardiovascular: Chest pain (8%), chest discomfort (6%), vasodilatation (≥5%), peripheral edema (1% to ≤4%)

Central nervous system: Headache (1% to 16%), dizziness (5% to 6%), chills (≤5%)

Dermatologic: Urticaria (1% to 5%), pruritus (1% to 4%)

Endocrine & metabolic: Hot flash (5%)

Gastrointestinal: Nausea (1% to 11%), oral candidiasis (5%), sore throat (≥5%)

Genitourinary: Urinary tract infection (13%)

Hematologic: Hemorrhage (≥5%)

Hepatic: Increased liver enzymes (6%)

Local: Bruising at injection site (8%)

Neuromuscular & skeletal: Musculoskeletal discomfort (16%), back pain (5%), weakness (≥5%)

Respiratory: Upper respiratory tract infection (2% to 16%), cough (5% to 9%), rhinorrhea (4% to 6%), bronchitis (≥5%), rhinitis (≥5%), sinusitis (≥5%), dyspnea (5%)

Miscellaneous: Fever (1% to ≥5%)

Postmarketing and/or case reports: Abdominal pain, asthma, back pain, confusion, diarrhea, drowsiness, flu-like symptoms, flushing, hypersensitivity reaction, hypoesthesia, hypoxia, injection site reaction, paresthesia, skin rash (exfoliative, generalized), syncope, tachycardia, visual disturbance, vomiting

Warnings/Precautions

Concerns related to adverse effects:

• Hypersensitivity: Severe hypersensitivity and anaphylactic reactions may occur; stop infusion promptly for acute hypersensitivity; immediate treatment (including epinephrine and/or other supportive therapy) should be available. May contain trace amounts of IgA; severe anaphylaxis may occur in patients with anti-IgA antibody; use in patients with selective IgA deficiency with known anti-IgA antibody is contraindicated

Disease-related concerns:

• Fluid overload: Plasma volume may increase following infusion; use with caution in patients at risk for fluid overload.

Dosage form specific issues:

• Human plasma: Product of human plasma; may potentially contain infectious agents which could transmit disease. Screening of donors, as well as testing and/or inactivation or removal of certain viruses, reduces the risk. Infections thought to be transmitted by this product should be reported to the manufacturer.

Monitoring Parameters

Alpha1-PI serum levels; lung function; vital signs during infusion

Pregnancy Risk Factor

C

Pregnancy Considerations

Animal reproduction studies have not been conducted.

Patient Education

• Discuss specific use of drug and side effects with patient as it relates to treatment. (HCAHPS: During this hospital stay, were you given any medicine that you had not taken before? Before giving you any new medicine, how often did hospital staff tell you what the medicine was for? How often did hospital staff describe possible side effects in a way you could understand?)

• Patient may experience headache, signs of common cold, sinus pain, loss of strength and energy, muscle pain, nausea, rhinorrhea, or injection site irritation. Have patient report immediately to prescriber signs of parvovirus B19 or hepatitis A infection (chills, severe fatigue, rhinorrhea, rash, joint pain, lack of appetite, nausea, vomiting, abdominal pain, or jaundice), angina, severe dizziness, passing out, or shortness of breath (HCAHPS).

• Educate patient about signs of a significant reaction (eg, wheezing; chest tightness; fever; itching; bad cough; blue skin color; seizures; or swelling of face, lips, tongue, or throat). Note: This is not a comprehensive list of all side effects. Patient should consult prescriber for additional questions.

Intended Use and Disclaimer: Should not be printed and given to patients. This information is intended to serve as a concise initial reference for healthcare professionals to use when discussing medications with a patient. You must ultimately rely on your own discretion, experience and judgment in diagnosing, treating and advising patients.

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