AI Summary A1-PI therapy (Prolastin-C, Glassia, Aralast NP, or Zemaira) is dosed at 60 mg/kg by weekly IV infusion and is not indicated for AATD-related liver disease.
Medically reviewed.
Alpha1-proteinase inhibitor (A1-PI) augmentation therapy — sold under the brand names Prolastin-C, Glassia, Aralast NP, and Zemaira — is a plasma-derived medication used to treat adults with severe Alpha-1 Antitrypsin Deficiency (AATD) and clinically significant emphysema. These therapies replace the missing or deficient A1-PI protein to help protect lung tissue from ongoing damage caused by neutrophil elastase. Although AATD can also affect the liver, A1-PI augmentation therapy is not indicated for treating AATD-related liver disease. Treatment decisions should always be individualized and guided by a healthcare professional.
This page covers A1-PI therapy's role in AATD management — how it works, who may be eligible, benefits, side effects, and monitoring — and clarifies why it targets lung disease rather than liver disease. You can also connect with a liver specialist near you for personalized guidance on managing AATD-related liver health.
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ABOUT A1-PI TREATMENT
Alpha1-proteinase inhibitor (A1-PI) is a plasma-derived augmentation therapy used to treat adults with severe Alpha-1 Antitrypsin Deficiency (AATD) and clinically significant emphysema. It is sold under several brand names — Prolastin-C, Glassia, Aralast NP, and Zemaira — which this page refers to collectively as A1-PI therapy.
AATD is a genetic disorder in which the liver produces an abnormally shaped alpha-1 antitrypsin protein. This misshapen protein cannot be released properly into the bloodstream, so too little of it reaches the lungs to protect them, while excess protein builds up inside liver cells.
A1-PI therapy works by replacing the missing or deficient alpha1-proteinase inhibitor protein in the blood, which helps protect lung tissue from ongoing damage caused by neutrophil elastase. Although AATD can also affect the liver, A1-PI therapy does not correct or treat the liver accumulation of abnormal protein — it is not indicated for AATD-related liver disease. It is typically used alongside pulmonary rehabilitation, avoidance of respiratory irritants, and regular monitoring by your healthcare provider.
WHO A1-PI MAY BE SUITABLE FOR
Eligibility for A1-PI therapy depends on your diagnosis, disease severity, and individual health needs. Your healthcare provider may prescribe A1-PI therapy if you have severe AATD with emphysema, if blood testing confirms significantly reduced alpha1-proteinase inhibitor levels, if you require long-term augmentation therapy to slow the progression of lung disease, and if you are under specialist care for AATD-related pulmonary disease.
Because A1-PI therapy addresses lung disease and does not treat AATD-related liver disease, people whose main concern is liver involvement — such as elevated liver enzymes, fibrosis, or cirrhosis linked to AATD — need a different care plan focused on liver monitoring and management. Your medical history, current health status, and other medications all factor into whether A1-PI therapy is an appropriate option for you.
Because every patient's health profile is different, there is no one-size-fits-all answer to who should receive A1-PI therapy. Treatment decisions are individualized and should be made by a qualified healthcare professional, typically a pulmonologist, after a full assessment of your lung and liver condition and treatment goals.
ELIGIBILITY OVERVIEW
A1-PI therapy may be considered for adults with the following conditions, risk factors, or treatment goals:
Before recommending A1-PI therapy, healthcare providers evaluate several factors to determine whether it is an appropriate option, including:
HOW A1-PI CAN HELP YOUR LUNGS
A1-PI therapy works by replacing the deficient A1-PI protein directly in the bloodstream. Once infused, the protein circulates to the lungs, where it inhibits neutrophil elastase — an enzyme released during inflammation that, left unchecked, breaks down the elastic tissue (alveolar structures) that keeps lung airways open.
By restoring A1-PI levels, this therapy helps protect alveolar structures from progressive destruction and supports long-term preservation of lung function. Over time, this can help slow the decline in lung density associated with AATD.
It is important to understand what A1-PI therapy does not do: it does not correct or treat the accumulation of abnormal alpha-1 protein inside liver cells, and it is not indicated for AATD-related liver disease. For patients with AATD who also have liver involvement, liver health is managed separately, typically through regular monitoring with Liver Function Tests (LFTs) and guidance from a liver specialist.
LUNG BENEFITS
A1-PI therapy replaces the deficient alpha1-proteinase inhibitor protein in the bloodstream, restoring levels needed to protect lung tissue.
Restored A1-PI inhibits neutrophil elastase activity in the lungs, reducing ongoing enzymatic damage to alveolar structures.
By protecting alveolar structures, A1-PI therapy helps slow the decline in lung density and supports long-term preservation of pulmonary function.
A1-PI therapy works to protect the lungs from ongoing damage in severe AATD. It does not treat AATD-related liver disease, so liver health needs its own monitoring plan. Therapy should only be started and monitored under the guidance of a qualified healthcare professional.
TREATMENT PROCESS
A1-PI products are available as intravenous formulations — either a ready-to-use liquid or a lyophilized powder that requires reconstitution, depending on the specific product.
Standard dosing is 60 mg/kg administered intravenously once weekly. Your healthcare provider determines the right dose for you.
A1-PI therapy is given as a slow intravenous infusion, in a healthcare setting or via trained home infusion services. It is not a pill or a subcutaneous injection. Infusion rates and preparation steps may vary slightly by product.
A1-PI therapy is typically long-term and often lifelong, continued as long as clinical benefit is maintained and the treatment is well tolerated.
Store refrigerated at 2°C-8°C (36°F-46°F). Do not freeze. Protect from light until use; product-specific variations may apply.
Your healthcare provider will regularly monitor lung function, infusion-related reactions, and overall clinical response. Periodic reassessment by a pulmonology specialist is advised. Report any side effects or unusual symptoms to your healthcare provider.
BENEFITS AND SAFETY
A1-PI therapy may support people with severe Alpha-1 Antitrypsin Deficiency and emphysema by restoring deficient A1-PI protein levels in the bloodstream, which helps reduce ongoing neutrophil elastase-mediated lung tissue damage. This can help slow the progression of emphysema and support long-term preservation of lung function in appropriate patients. The extent of these benefits varies from person to person, depending on lung condition, overall health, and how the body responds to therapy. A1-PI therapy is not a stand-alone treatment — it works best as part of a broader, personalised care plan that combines weekly infusions with pulmonary rehabilitation, smoking cessation where applicable, and ongoing monitoring of lung function.
As with any medication, A1-PI therapy may cause side effects, ranging from mild symptoms to infusion-related reactions. Because it does not treat AATD-related liver disease, your healthcare provider will also coordinate separate monitoring of liver health if needed, and will assess whether A1-PI therapy remains a suitable option for you throughout treatment.
BENEFITS
A1-PI therapy restores deficient alpha1-proteinase inhibitor levels in the bloodstream, directly addressing the underlying protein deficiency in severe AATD.
Restored A1-PI levels help reduce neutrophil elastase-mediated lung tissue damage, protecting alveolar structures from progressive destruction.
By slowing ongoing lung tissue damage, A1-PI therapy supports disease stabilization and helps slow progression of emphysema in appropriate patients with AATD.
Weekly infusions support ongoing maintenance of lung function over the long term, making A1-PI therapy suitable for patients who need sustained protection.
These effects are often more noticeable around the time of infusion and may improve over time. Your healthcare provider will discuss the potential benefits and risks before treatment and provide ongoing support to ensure safe use.
SUPPORTING YOUR HEALTH
A1-PI therapy works best alongside lifestyle changes that protect both lung and liver health. Smoking cessation, pulmonary rehabilitation, and avoiding respiratory irritants help preserve lung function, while vaccinations reduce infection risk.
Because AATD can also affect the liver, a liver-friendly diet and regular monitoring remain important even though A1-PI therapy itself does not treat liver involvement.
Habits that may support treatment:
COMMON QUESTIONS
No. A1-PI (alpha1-proteinase inhibitor) augmentation therapy is not indicated for treating AATD-related liver disease. It replaces the deficient protein in the bloodstream to protect the lungs from neutrophil elastase damage, but it does not correct or remove the abnormal protein that accumulates inside liver cells. If you have AATD and liver involvement, your provider will manage your liver health with separate monitoring and treatment.
Prolastin-C, Glassia, Aralast NP, and Zemaira are all plasma-derived A1-PI products used to treat the same condition — severe AATD with clinically significant emphysema — at the same standard dose of 60 mg/kg weekly by intravenous infusion. They differ mainly in formulation (ready-to-use liquid versus lyophilized powder requiring reconstitution) and minor variations in infusion rate and preparation steps. Your healthcare provider will determine which product is right for you.
A1-PI therapy is given as a slow intravenous infusion, typically once weekly at a standard dose of 60 mg/kg. It can be administered in a healthcare setting or through trained home infusion services, depending on your provider's recommendation and your comfort with home infusion.
A1-PI therapy is typically long-term and often lifelong. It is continued as long as it provides clinical benefit and is well tolerated. Do not stop treatment without first talking to your healthcare provider, since discontinuing therapy may allow lung damage to progress.
A1-PI therapy is generally reserved for adults with severe AATD and clinically significant emphysema, confirmed by blood testing showing significantly reduced A1-PI levels. It is not appropriate for people whose main health concern is AATD-related liver disease, since it does not treat liver involvement. A pulmonologist or AATD specialist determines eligibility after a full assessment of your lung function and overall health.
Common side effects include headache, fatigue, dizziness, upper respiratory tract infections, and infusion-related reactions such as fever, chills, or discomfort during or after the infusion. These effects are often more noticeable around the time of infusion and may improve over time. Report any side effects to your healthcare provider.
Medical disclaimer: LiverDirectory provides educational information and a directory of providers. It does not provide medical advice, diagnosis, or treatment. Always consult a licensed physician regarding any medical condition.
