Alpha-1 Proteinase Inhibitor

How it works

It supplements deficient circulating alpha-1 antitrypsin, restoring protease-antiprotease balance and limiting neutrophil-elastase-mediated destruction of lung tissue.

Prescribing in practice

• As a human plasma-derived product it carries a theoretical risk of transmissible infection and may cause hypersensitivity reactions, so administer where these can be managed.
• It is given by intravenous infusion, typically on a regular scheduled basis.
• Use is restricted to confirmed severe deficiency with demonstrable lung disease rather than deficiency alone.

Monitoring

Monitor for infusion-related and hypersensitivity reactions during administration and review respiratory status over time.

Counselling the patient

• This treatment replaces a protein you are missing and is given by a drip.
• Report rash, fever, breathlessness or other reactions during or after infusion.
• Smoking cessation remains essential to protect your lungs.

Evidence & guidelines

Augmentation therapy is supported in confirmed severe alpha-1 antitrypsin deficiency per specialist respiratory guidance and the SPC.