Prolastin Elastin
Also known as: Alpha-1 Antitrypsin, AAT, Alpha-1 proteinase inhibitor, Prolastin
Overview
Alpha-1 antitrypsin (AAT) is a protein primarily produced in the liver, crucial for protecting the lungs from damage caused by neutrophil elastase, an enzyme released by white blood cells. AAT is naturally found in human plasma and serves as a protease inhibitor. Prolastin, a purified form of AAT derived from human plasma, is mainly used in augmentation therapy to treat emphysema associated with alpha-1 antitrypsin deficiency (AATD). AATD is a genetic condition characterized by low levels of AAT in the blood, leading to increased risk of lung damage. Treatment with Prolastin aims to increase AAT levels in the blood, thereby reducing the activity of neutrophil elastase and protecting lung tissue. It is administered intravenously to ensure direct delivery into the bloodstream. Research has well-established the efficacy of Prolastin in slowing the decline of lung function in AATD patients.
Benefits
The primary benefit of Prolastin is its ability to slow the decline in lung function in patients with alpha-1 antitrypsin deficiency (AATD). Augmentation therapy with Prolastin increases serum AAT levels, which helps to inhibit neutrophil elastase activity in the lungs. This is particularly beneficial for individuals with severe AATD and low plasma AAT levels (below 11 μM). Clinical trials have demonstrated that regular infusions of Prolastin can lead to a significant increase in serum AAT levels, although the clinical impact on lung function parameters may vary among individuals. While Prolastin increases AAT levels, studies indicate that it does not significantly alter sputum volume or bacteriology. The benefits of Prolastin are typically observed over long-term use, requiring consistent and regular infusions to maintain therapeutic levels.
How it works
Prolastin works by inhibiting neutrophil elastase, an enzyme that can damage lung tissue. In individuals with alpha-1 antitrypsin deficiency (AATD), the deficiency of AAT allows neutrophil elastase to degrade elastin, a crucial component of lung tissue, leading to emphysema. Prolastin, administered intravenously, directly increases the levels of AAT in the bloodstream. This augmented AAT then binds to and neutralizes neutrophil elastase, protecting the lung tissue from enzymatic damage. The primary biological pathway involves the inhibition of neutrophil elastase activity, thereby preserving the structural integrity of the respiratory system.
Side effects
Prolastin is generally considered safe when administered appropriately, but it is associated with several potential side effects. Common side effects include chills and arthralgia (joint pain). Uncommon and rare side effects are not well-documented in the available literature, but serious adverse events are infrequent. Contraindications for Prolastin include hypersensitivity to AAT or any other components of the product. Caution is advised in patients with known hypersensitivity reactions. While no significant drug interactions have been reported, it is essential to inform healthcare providers of all medications being taken. Patients should be monitored for hypersensitivity reactions during and after Prolastin infusions. Overall, the safety profile of Prolastin is well-established, but vigilance regarding potential adverse reactions is necessary.
Dosage
The typical minimum effective dose of Prolastin is 60 mg/kg administered once weekly via intravenous infusion. This standard dosing regimen aims to maintain therapeutic levels of AAT in the bloodstream. While the maximum safe dose is not definitively established, the dosage is generally determined by clinical need and patient tolerance. Regular infusions are necessary to sustain therapeutic AAT levels. Direct intravenous delivery ensures high bioavailability, bypassing any absorption-related factors. There are no specific required cofactors for Prolastin administration. It is crucial to adhere to the prescribed dosing schedule to achieve optimal therapeutic outcomes and minimize the risk of lung function decline in AATD patients.
FAQs
How often do I need Prolastin infusions?
Prolastin is typically administered intravenously once weekly to maintain therapeutic levels of alpha-1 antitrypsin in the blood. Regular infusions are crucial for managing AATD.
What are the expected results of Prolastin treatment?
Prolastin treatment aims to slow the decline in lung function associated with alpha-1 antitrypsin deficiency. It helps protect lung tissue from damage caused by neutrophil elastase.
Is Prolastin a cure for alpha-1 antitrypsin deficiency?
No, Prolastin is not a cure for AATD. It is a management therapy that helps alleviate symptoms and slow disease progression by increasing AAT levels in the blood.
Are there any safety concerns with Prolastin?
Hypersensitivity reactions are a potential concern. Patients should be monitored during and after infusions. Common side effects include chills and joint pain, but serious adverse events are rare.
How is Prolastin administered?
Prolastin is administered intravenously by a healthcare professional. The standard dosing is 60 mg/kg, given once weekly to maintain adequate AAT levels.
Research Sources
- https://www.tandfonline.com/doi/pdf/10.1080/15412550902905961 – This meta-analysis supports the use of augmentation therapy in patients with α1-AT deficiency to slow the decline in lung function. The study compiles data from multiple sources to strengthen the evidence for the effectiveness of Prolastin in managing AATD.
- https://www.tga.gov.au/sites/default/files/auspar-prolastin-c-171011-cer.docx – A clinical trial using Prolastin demonstrated an increase in serum AAT levels in patients. However, the study found no significant changes in lung function parameters or sputum bacteriology, indicating that while AAT levels increase, the clinical impact may vary.
- https://pmc.ncbi.nlm.nih.gov/articles/PMC5529111/ – This review highlights the clinical utility of alpha-1 proteinase inhibitor in managing AATD. It notes the importance of maintaining stable trough serum levels and anti-neutrophil elastase capacity to effectively protect lung tissue.
- https://pmc.ncbi.nlm.nih.gov/articles/PMC2726081/ – This article discusses the pathophysiology, diagnosis, and treatment of alpha-1 antitrypsin deficiency (AATD). It provides an overview of the genetic basis of AATD and the role of AAT in protecting the lungs from damage, as well as the use of augmentation therapy to increase AAT levels in the blood.
- https://www.aetna.com/cpb/medical/data/100_199/0145.html – This clinical policy bulletin assesses the use of alpha-1 proteinase inhibitor for AATD. It reviews the evidence supporting its efficacy in slowing lung function decline and provides guidelines for appropriate patient selection and monitoring.
