How Does Alpha-1 Proteinase Inhibitor Work?

Reviewed on 12/29/2021

How does alpha-1 proteinase inhibitor work?

Alpha-1 proteinase inhibitor is a medication used for treating alpha-1 proteinase inhibitor deficiency, also known as alpha-1 antitrypsin deficiency, an inherited condition that affects the lungs and liver. Alpha-1 proteinase inhibitor is a purified substance derived from plasma from human donors.

During lung inflammations, immune cells neutrophils, and macrophages produce neutrophil elastase, an enzyme that destroys bacteria, but also damages the elastic tissue in the lungs and stimulates mucus secretion. Alpha-1 proteinase inhibitor, a protein produced by the liver, protects lung tissue damage by inhibiting the activity of neutrophil elastase.

In people with alpha-1 antitrypsin deficiency, neutrophil elastase regulation is impaired, leading to elastic damage to the lungs and consequently, emphysema, a lung disease that affects airflow and gas exchange in the lungs. A purified human alpha-1 proteinase inhibitor is used to supplement the natural deficiency in the protein.

How is alpha-1 proteinase inhibitor used?

Alpha-1 proteinase inhibitor is administered as IV infusions to treat the following conditions:


  • Alpha-1 antitrypsin deficiency

Orphan designation:


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What are side effects of alpha-1 proteinase inhibitor?

Side effects of alpha-1 proteinase inhibitor may include the following:

Information contained herein is not intended to cover all possible side effects, precautions, warnings, drug interactions, allergic reactions, or adverse effects. Check with your doctor or pharmacist to make sure these drugs do not cause any harm when you take them along with other medicines. Never stop taking your medication and never change your dose or frequency without consulting your doctor.

What are names of some alpha-1 proteinase inhibitor drugs?

Generic and brand names of alpha-1 proteinase inhibitor drugs include:


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