A1-Proteinase Inhibitor (Human)

Name: A1-Proteinase Inhibitor (Human)


A naturally occurring serine protease inhibitor.1 2 3 11 15

Uses for a1-Proteinase Inhibitor (Human)

Congenital α1-Proteinase Inhibitor Deficiency

Replacement therapy in patients with congenital α1-proteinase inhibitor (also called α1-antitrypsin) deficiency and clinically evident emphysema.1 2 3 7 8 11 15

Not indicated as therapy for patients with lung disease in whom congenital α1-proteinase inhibitor deficiency has not been established.1 3 11

The American Thoracic Society and the European Respiratory Society (ATS/ERS) state that α1-proteinase inhibitor therapy does not confer benefit in, and is not recommended for, patients who have α1-proteinase-associated liver disease.a

For the Consumer

Applies to alpha 1-proteinase inhibitor: intravenous powder for solution, intravenous solution

Along with its needed effects, alpha 1-proteinase inhibitor may cause some unwanted effects. Although not all of these side effects may occur, if they do occur they may need medical attention.

Check with your doctor or nurse immediately if any of the following side effects occur while taking alpha 1-proteinase inhibitor:

More common
  • Bladder pain
  • bloody or cloudy urine
  • body aches or pain
  • chills
  • cough
  • difficult, burning, or painful urination
  • difficulty with breathing
  • ear congestion
  • fever
  • frequent urge to urinate
  • headache
  • loss of voice
  • lower back or side pain
  • nasal congestion
  • runny nose
  • sneezing
  • sore throat
  • unusual tiredness or weakness
  • Bloating or swelling of the face, arms, hands, lower legs, or feet
  • blurred vision
  • chest pain
  • cough producing mucus
  • diarrhea
  • difficult or labored breathing
  • dizziness
  • feeling faint, dizzy, or lightheadedness
  • flushing or redness of the skin, especially on the face and neck
  • general feeling of discomfort or illness
  • joint pain
  • loss of appetite
  • muscle aches and pains
  • nausea
  • nervousness
  • noisy breathing
  • pounding in the ears
  • rapid weight gain
  • shivering
  • slow or fast heartbeat
  • sweating
  • tightness in the chest
  • tingling of the hands or feet
  • trouble sleeping
  • unusual weight gain or loss
  • vomiting
Incidence not known
  • Confusion
  • difficulty with swallowing
  • dizziness, faintness, or lightheadedness when getting up suddenly from a lying or sitting position
  • fast, pounding, or irregular heartbeat or pulse
  • hives
  • itching, puffiness or swelling of the eyelids or around the eyes, face, lips, or tongue
  • skin rash

Some side effects of alpha 1-proteinase inhibitor may occur that usually do not need medical attention. These side effects may go away during treatment as your body adjusts to the medicine. Also, your health care professional may be able to tell you about ways to prevent or reduce some of these side effects. Check with your health care professional if any of the following side effects continue or are bothersome or if you have any questions about them:

More common
  • Feeling of warmth
  • itching skin
  • muscle or bone pain
  • pain or tenderness around the eyes and cheekbones
  • redness of the face, neck, arms, and occasionally, upper chest
  • sudden sweating
  • Back pain
  • bloating
  • change in taste
  • changes in vision
  • fever
  • hives or welts
  • loss of taste
  • pain
  • redness of the skin
  • sleepiness or unusual drowsiness
  • swelling of the joints
  • unusual drowsiness, dullness, tiredness, weakness, or feeling of sluggishness

Usual Adult Dose for Alpha-1 Proteinase Inhibitor Deficiency

Chronic augmentation and maintenance therapy in patients with clinical evidence of emphysema: 60 mg/kg via IV infusion once a week

Infusion rates:
Aralast(TM), Aralast NP(TM), Prolastin(R), Prolastin(R)-C, Zemaira(R): Should be administered at a rate not exceeding 0.08 mL/kg per minute
Glassia(TM): Should be administered at a rate not exceeding 0.04 mL/kg per minute


Alpha 1-proteinase inhibitor is contraindicated in individuals with selective IgA deficiencies who have known antibodies against IgA. Such individuals may experience severe reactions, including anaphylaxis, to IgA, which may be present in alpha 1-proteinase inhibitor preparations.

The rate of administration should be monitored closely, at least until the physician has had sufficient experience with a given patient. Vital signs should be monitored continuously and the patient should be carefully observed throughout the infusion. If anaphylactic or severe anaphylactoid reactions occur, the infusion should be discontinued immediately. Epinephrine and other appropriate supportive therapy should be available for the treatment of any acute anaphylactic or anaphylactoid reaction.

Alpha 1-proteinase inhibitor should be used with caution in patients at risk for circulatory overload. As with any colloid solution, there may be an increase in plasma volume following IV administration.

Safety and effectiveness have not been established in pediatric patients (less than 18 years of age).

Other Comments

Aralast(TM), Aralast NP(TM), Prolastin(R), Prolastin(R)-C, and Zemaira(R) should be administered within 3 hours following reconstitution. Drug and diluent should be at room temperature before reconstitution.

Glassia(TM) should be administered at room temperature within 3 hours of entering the vials.