People with alpha 1 antitrypsin deficiency usually develop the first signs and symptoms of lung disease between ages 20 and 50. The individual is diagnosed with congenital alpha 1 antitrypsin deficiency. Indefinite authorizationmay be granted for members prescribed an alpha. Alpha1 antitrypsin aat is an acutephase reactant protein that. This product profiler introduces health care professionals to alpha 1proteinase inhibitor human, zemaira, a u. Alpha 1proteinase inhibitor human aralasto solvent detergent treated nanofiltered for intravenous use only product information description alpha 1proteinase inhibitor human, aralast. Aat deficiency is a genetic disorder in which abnormally low levels of the protein aat allow certain enzymes to attack healthy tissues, especially the lungs. Prior authorization form for alpha1 proteinase inhibitors.
Zemaira alpha1proteinase inhibitor patient website. Diagnosis of congenital alpha1 antitrypsin deficiency confirmed by one of the following. A low concentration of aat is associated with slowly progressive, moderateto. Pdf expression of alpha1 proteinase inhibitor in human. The earliest symptoms are shortness of breath following mild. May 27, 2019 alpha 1 proteinase inhibitor is used to treat alpha 1 antitrypsin deficiency in people who have symptoms of emphysema. Human alpha1proteinase inhibitor is a wellcharacterized protease inhibitor with a wide spectrum of anti protease activity. Uterine uptake of alpha 2macroglobulin and alpha 1 proteinase inhibitor from the blood during early implantation in the mouse. And patient has an fev 1 in the range of 3065% of predicted.
Food and drug administration fdaapproved indications 1. Alpha1proteinase inhibitor memorial sloan kettering cancer. Deficiency of alpha 1 proteinase inhibitor a 1 pi, also known as alpha 1 antitrypsin aat deficiency is a chronic, hereditary disorder, characterized by reduced levels of a1pi in the blood and lungs. Hypersensitivity to alpha1 proteinase inhibitor patients with selective iga deficiencies iga less than 15 mgdl who have known antibody against iga antiiga antibody not approved if. Individual is 18 years of age or older treatment of congenital alpha1antitrypsin deficiencyassociated lung disease alpha 1. Alpha1 proteinase inhibitor human intravenous route. Page 3 of 5 alpha1 proteinase inhibitors are not indicated as therapy for patients with lung disease in whom congenital alpha1 proteinase inhibitor deficiency has not been established. Longterm clinical outcomes following treatment with alpha 1.
Ne is released by activated neutrophils and can degrade elastin within connective. Pdf human alpha1proteinase inhibitor is a wellcharacterized protease inhibitor with a wide spectrum of antiprotease activity. Uterine uptake of alpha 2macroglobulin and alpha 1proteinase inhibitor from the blood during early implantation in the mouse. The total protein content is approximately 1,100 mg per vial. Alpha 1 antitrypsin deficiency is an inherited disorder that may cause lung disease and liver disease. Alpha 1 proteinase inhibitor human, zemaira is indicated to raise the plasma level of alpha 1 proteinase inhibitor a 1 pi in patients with a 1 pi deficiency and related emphysema. Us20110237781a1 method of preparing alpha1 proteinase. Alpha 1 proteinase inhibitor may also be used for purposes not listed in this medication guide. An important function of a 1 pi in the lung is to inhibit neutrophil elastase, one of various proteolytic enzymes released by activated neutrophils during inflammation. Codes referenced in this clinical policy are for informational purposes only. Food and drug administrationapproved treatment indicated for chronic augmentation and maintenance therapy in individuals with alpha 1proteinase inhibitor alpha 1pi deficiency and clinical evidence of emphysema. Clinical utility of alpha 1 proteinase inhibitor in the management of adult patients with severe alpha 1 antitrypsin deficiency. This protein occurs naturally in the body and is important for preventing the breakdown of tissues in the lungs. Alpha 1 proteinase inhibitor human aralasto solvent detergent treated nanofiltered for intravenous use only product information description alpha 1 proteinase inhibitor human, aralast.
Alpha 1 antitrypsin aat is one of the major serum proteins in the circulation. The spectrum of aatdrelated disease and the age at clinical onset is quite broad. Studies have not demonstrated alpha 1 proteinase inhibitor to be effective for this condition. Alpha 1 antitrypsin deficiency aatd is an autosomal recessive genetic disorder that results in decreased. Respreeza, innhuman alpha1proteinase inhibitor european. Its major physiological role is inhibition of neutrophil elastase in. Alpha 1proteinase inhibitor is a protein, also called alpha 1antitrypsin. Alpha1proteinase inhibitors commercial medical benefit. Proteases are enzymes which must be carefully regulated, otherwise they can attack and damage normal tissue. Onset of lung problems is typically between 20 and 50 years old. Introduction to alpha1 antitrypsin deficiency aatd and currently available treatment options. Initial approval criteria alpha 1 proteinase inhibitors are considered medically necessary for emphysema secondary to alpha 1 antitrypsin att deficiency when the following criteria are met. Specialty guideline management alpha1proteinase inhibitors. It is mostly produced by hepatocytes, 1 although other cells can also express aat including epithelial cells, 2,3 monocytes, 4 macrophages, 5 neutrophils, 6 cornea 7 and pancreatic islet cells.
Alpha1proteinase inhibitor memorial sloan kettering. Numbers of patients are 75 for a1pi earlystart group and 64 for placebo delayedstart group. Prolastin is prepared from pooled human plasma of normal donors by. Prolastin is intended for use in therapy of congenital alpha1antitrypsin deficiency. Medical necessity criteria page 1 4 alpha 1 proteinase inhibitors. Find patient medical information for alpha 1 proteinase inhibitor human intravenous on webmd including its uses, side effects and safety, interactions, pictures, warnings and user ratings. Alpha 1 antitrypsin deficiency a1ad or aatd is a genetic disorder that may result in lung disease or liver disease.
Alpha 1proteinase inhibitors page 3 of 6 unitedhealthcare oxford clinical policy effective 11012019 19962019, oxford health plans, llc background deficiency of alpha 1proteinase inhibitor a 1pi, also known as alpha 1antitrypsin deficiency, is characterized by reduced levels of a 1pi in the blood and lungs. If you have an allergy to alpha 1proteinase inhibitor or any other part of this drug. Alpha 1 proteinase inhibitor injection, also called alpha 1 pi, is used to treat a certain type of emphysema a lung condition. Alpha 1proteinase inhibitor is a protein, also called alpha 1 antitrypsin.
Alpha1antitrypsin deficiency usage of alpha1proteinase inhibitor concentrate in replacement therapy 1 november 1986 san diego, california james e. Jun 03, 2019 detailed alpha 1 proteinase inhibitor dosage information for adults. Lists contain the alpha1 proteinase inhibitor products with supporting documents. Longterm clinical outcomes following treatment with alpha. Alpha 1proteinase inhibitor dosage guide with precautions. Revised 12019 page 1 of 1 prior authorization form for alpha1 proteinase inhibitors alpha 1proteinase inhibitor human aralast np alpha 1proteinase inhibitor human glassia alpha 1proteinase inhibitor human prolastin c alpha 1proteinase inhibitor human zemaira. Recommended authorization criteria coverage of prolastinc, aralast np, zemaira, or glassia is recommended in those who meet the following criteria. Respreeza 4,000 mg powder and solvent for solution for infusion. Bronchiectasis without alpha 1 antitrypsin deficiency. This may result in shortness of breath, wheezing, or an increased risk of lung infections.
Prolastinc alpha1 proteinase inhibitor zemaira alpha1 proteinase inhibitor approval criteria requests for alpha1 proteinase inhibitor therapy may be approved if the following criteria are met. Clinical utility of alpha1 proteinase inhibitor in the management of. Alpha1 proteinase inhibitor prolastinc is a prescription medicine used to treat alpha 1 antitrypsin aat deficiency. Global initiative for chronic obstructive lung disease gold. Alpha 1 proteinase inhibitor is a protein, also called alpha 1 antitrypsin. Medical necessity criteria page 14 alpha1proteinase inhibitors. Alpha 1proteinase inhibitors page 3 of 6 unitedhealthcare oxford clinical policy effective 11012019 19962019, oxford health plans, llc background deficiency of alpha 1 proteinase inhibitor a 1 pi, also known as alpha 1 antitrypsin deficiency, is characterized by reduced levels of a 1 pi in the blood and lungs. In people who lack the alpha 1 antitrypsin protein, breakdown of lung tissues can. Emphysema due to alpha1antitrypsin aat deficiency patient is 18 years or older. Includes dosages for alpha 1 proteinase inhibitor deficiency. Glassia alpha1 proteinase inhibitor human, intravenous. Alpha 1proteinase inhibitor human, zemaira is indicated to raise the plasma level of alpha 1proteinase inhibitor a 1pi in patients with a 1pi deficiency and related emphysema. Alpha1 antitrypsin and autoimmunity journal of the copd.
Alpha1 antitrypsin deficiency genetics home reference nih. Treatment of alpha1 antitrypsin deficiency uptodate. Moreover, the drug 5aminosalicylic acid prevented the inactivation of alpha 1 proteinase inhibitor by. Prior authorization is recommended for prescription benefit coverage of an alpha 1 proteinase inhibitor. Pdf uterine uptake of alpha 2macroglobulin and alpha 1. Above criteria are not met emphysema is due to environmental triggers emphysema is caused by tobacco use. Alpha 1proteinase inhibitor is used to treat alpha 1 antitrypsin deficiency in people who have symptoms of emphysema. Since the end of the 1980s augmentation therapy with alpha1 antitrypsin aat from human plasma has been available for specific treatment of emphysema due to aat deficiency. The signs and symptoms of the condition and the age at which they appear vary among individuals. Find patient medical information for alpha1proteinase inhibitor human intravenous on webmd including its uses, side effects and safety, interactions, pictures, warnings and user ratings. Alpha1 proteinase inhibitors aralast np, glassia, prolastinc, zemaira alpha1 proteinase inhibitors fep clinical rationale aralast np, glassia, prolastinc, and zemaira are intravenous infusions indicated for individuals with clinically evident emphysema due to severe deficiency of alpha 1 pi, also known as alpha 1 antitrypsin aat deficiency. In people who lack the alpha 1antitrypsin protein, breakdown of lung tissues can lead to emphysema damage to the air sacs in the lungs.
Expression of alpha 1 proteinase inhibitor in human islet microvascular endothelial cells. Alpha 1 antitrypsin deficiency is a genetic inherited disorder and alpha 1 proteinase inhibitor will not cure this condition. Alpha 1 proteinase inhibitor human full prescribing information 1 indica tions and usage zemaira is an alpha 1 proteinase inhibitor a 1 pi indicated for chronic augmentation and maintenance therapy in adults with a 1 pi deficiency and clinical evidence of emphysema. Indications the indications below including fdaapproved indications and compendial uses are considered a covered benefit provided that all the approval criteria are met and the member has no exclusions to. Alpha 1 antitrypsin deficiency aatd is a rare genetic disease characterized by low serum levels of alpha 1 03 proteinase inhibitor a1pi, also known as alpha 1 antitrypsin. Alpha1proteinase inhibitors infusion therapy emblemhealth. Zemaira increases antigenic and functional antineutrophil elastase. Alpha 1 proteinase inhibitor prolastinc is a prescription medicine used to treat alpha 1 antitrypsin aat deficiency. Also known as alpha 1 proteinase inhibitor, it blocks the destructive action of trypsin and elastin as well as several other proteases. In the scientific literature, the disorder is referred to as aat deficiency whereas the deficiency or replacement protein is referred to as alpha 1proteinase inhibitor. Detailed alpha 1proteinase inhibitor dosage information for adults. Alpha 1 antitrypsin aat, produced in the liver, is the most abundant of these serum protease inhibitors. Pdf heme binding to human alpha1 proteinase inhibitor.
May 30, 2017 alpha 1 antitrypsin deficiency aatd is a common hereditary disorder caused by mutations in the serpina1 gene, which encodes alpha 1 antitrypsin aat. Alpha1antitrypsin deficiency usage of alpha1proteinase. Diagnosis of congenital alpha1antitrypsin deficiency confirmed by one of the following. Alpha 1 antitrypsin deficiency aatd, also known as alpha 1 proteinase inhibitor deficiency, is a genetic condition that leads to increased risk of lung and liver disease and several other conditions. The member displays clinically evident emphysema ii. Human alpha 1 proteinase inhibitor is a wellcharacterized protease inhibitor with a wide spectrum of antiprotease activity. Alpha1 antitrypsin deficiency clinical practice guidelines. The proteinase inhibitor, alpha 1 antitrypsin aat is predominantly produced in the liver1 and helps to regulate proteases2. Alpha1proteinase inhibitors aralast np, glassia, prolastinc. Prolastinc chronic augmentation and maintenance therapy in adults with clinical evidence of emphysema due to severe hereditary deficiency of alpha1 proteinase inhibitor alpha1antitrypsin deficiency 4. Review request for alpha 1 proteinase inhibitor therapy plan. Intravenous alpha 1 proteinase inhibitor will be used as augmentation addition to improve or complete therapy. This medicine replaces the protein when the body does not produce enough.
This policy may be updated and therefore subject to change. And patient has alpha1antitrypsin aat deficiency with pizz, piz null, or pi null, null phenotypes. Nov 09, 2018 alpha 1 proteinase inhibitor is a protein, also called alpha 1 antitrypsin. Intravenous alpha 1proteinase inhibitor human aralast np, glassia, prolastinc, zemaira is considered medically necessary when all of the following criteria are met. A protease inhibitor, it is also known as alpha 1 proteinase inhibitor a1pi or alpha 1 antiproteinase a1ap because it inhibits various proteases not just trypsin. The effect of this raised level on the frequency of pulmonary exacerbations and the progression of emphysema have not been established in clinical trials. The drug 5aminosalicylic acid rescues alpha 1proteinase. Alpha 1 antitrypsin deficiency aatd is a common hereditary disorder caused by mutations in the serpina1 gene, which encodes alpha 1 antitrypsin aat. The emphysema is caused by the lack of a protein called alpha 1 antitrypsin aat in the body. People with alpha1 antitrypsin deficiency usually develop the first signs and symptoms of lung disease between ages 20 and 50. Intravenous alpha 1 proteinase inhibitor human aralast np, glassia, prolastinc, zemaira is considered medically necessary when all of the following criteria are met. Clinical utility of alpha1 proteinase inhibitor in the. Alpha 1proteinase inhibitor may also be used for purposes not listed in this medication guide.
The members pretreatment serum aat level is less than 11 m. Alpha 1 antitrypsin also known as alpha 1 proteinase inhibitor is an antiprotease found in human plasma that inhibits the neutrophil elastase enzyme from degrading elastin tissues in the lung. Alpha1 antitrypsin deficiency a1ad or aatd is a genetic disorder that may result in lung disease or liver disease. Alpha1 proteinase inhibitors medical policy pdf blue cross blue. Alpha1 antitrypsin deficiency is an inherited disorder that may cause lung disease and liver disease. Original research the effect of alpha1 proteinase inhibitor. Individual has a diagnosis of congenital alpha 1 antitrypsin deficiency alpha1 proteinase inhibitor deficiency. Drug administration for manufacturing glassia alpha 1 proteinase inhibitor human, which is an intravenously administered biologic product indicated for chronic augmentation and maintenance therapy in individuals with emphysema due to congenital deficiency of alpha 1 proteinase inhibitor, also known as aat deficiency. Individual has a diagnosis of congenital alpha 1 antitrypsin deficiency alpha 1 proteinase inhibitor deficiency. Alpha 1 antitrypsin deficiency is a genetic inherited disorder and alpha 1proteinase inhibitor will not cure this condition. Page 3 of 5 alpha 1 proteinase inhibitors are not indicated as therapy for patients with lung disease in whom congenital alpha 1 proteinase inhibitor deficiency has not been established. Inhibitors and scavengers of neutrophilderived reactive oxygen species outlined the crucial role of hypochlorous acid in the alpha 1 proteinase inhibitor inactivation.
Intravenous alpha1 antitrypsin augmentation therapy for treating patients with alpha1 antitrypsin deficiency and lung disease. Karnaukhova and others published recombinant human alpha1proteinase inhibitor. Lung density was measured as adjusted pd15 gl per year. Aralast np, glassia, prolastinc, zemaira intravenous. Prolastinc alpha 1 proteinase inhibitor zemaira alpha 1 proteinase inhibitor approval criteria requests for alpha 1 proteinase inhibitor therapy may be approved if the following criteria are met. Background heme is a unique prosthetic group of various hemoproteins that perform diverse biological functions. This information from lexicomp explains what you need to know about this medication, including what its used for, how to take it, its side effects, and when to call your healthcare provider.