Alpha-1 proteinase inhibitor (human)
Alpha-1 antitrypsin replacement (augmentation therapy)
Alpha-1 antitrypsin replacement (augmentation therapy)
Purified human alpha-1 antitrypsin given by vein, for life, to slow emphysema in severe alpha-1 antitrypsin deficiency.
Also called: Alpha-1 antitrypsin, Alfa1 antitrypsin
Quick facts
- Group
- Alpha-1 antitrypsin replacement (augmentation therapy)
- Target
- Neutrophil elastase
- Used for
- Lifelong augmentation therapy
- Key effects
- Mild fever · Headache · Nausea
- Monitor
- Lung function and symptoms over time
- ATC
- B02AB02
No doses: the clinician sets them by the current guideline.
Mechanism of action
In severe deficiency the liver releases too little working alpha-1 antitrypsin into the blood, and neutrophil elastase released by white blood cells digests the elastic walls of the alveoli. Infusions of purified human alpha-1 antitrypsin raise the level of the protein in blood and lung. The protein traps and inactivates neutrophil elastase, which is meant to slow the loss of lung tissue.
- Deficiency leaves too little alpha-1 antitrypsin in blood and lung
- Neutrophil elastase digests alveolar elastin: emphysema
- Infused protein raises the level and traps the elastase
- Lung damage progresses more slowly
Indications
- Lifelong augmentation therapy in emphysema due to severe alpha-1 antitrypsin deficiency α1-antitrypsin deficiency →
Adverse effects
- Mild fever
- Headache
- Nausea
- Dizziness
Contraindications
- Not used for the liver disease of the deficiency: it is aimed at emphysema
- Not a substitute for stopping smoking
Key interactions
- Cigarette smoking
- Smoke damages deficient lungs faster; stopping smoking is essential
What is monitored
- Lung function and symptoms over time
- Reactions during and after each infusion
Administration and safety
- Given by intravenous infusion, usually every week or every four weeks
- Infusions continue for life
Pregnancy and breastfeeding
Data in pregnancy are limited; use is decided with the specialist.
Nursing care
- Check vital signs and watch for fever, headache, dizziness or allergic signs during the infusion
- Support stopping smoking and avoiding smoke and dust
- Teach that the therapy slows damage but does not reverse it
Pharmacokinetics
- Absorption
- Intravenous infusion only
Test yourself
Think of the answer, then tap the question to see it.
Which enzyme does infused alpha-1 antitrypsin inhibit?
Neutrophil elastase.
What is the aim of augmentation therapy?
To raise alpha-1 antitrypsin levels and slow lung damage in emphysema.
Which single measure matters most in a smoker with alpha-1 antitrypsin deficiency?
Stopping smoking.
Connections
← Back to the class: Alpha-1 antitrypsin replacement (augmentation therapy)
Educational content on how drugs work; not a prescribing guide and no substitute for a health professional's advice. No doses are given.
Sources: COPD - Alpha-1 Antitrypsin Deficiency | NHLBI, NIH (National Heart, Lung, and Blood Institute, 2026); Alpha-1 antitrypsin deficiency: MedlinePlus Medical Encyclopedia (MedlinePlus (U.S. National Library of Medicine), 2026); SERPINA1 gene: MedlinePlus Genetics (MedlinePlus (U.S. National Library of Medicine), 2026); ATCDDD - ATC/DDD Index (WHO Collaborating Centre for Drug Statistics Methodology, 2026).
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Representative model, for educational purposes only.