Alpha1proteinase inhibitors infusion therapy emblemhealth. The individual is diagnosed with congenital alpha1 antitrypsin deficiency. Alpha 1 proteinase inhibitor prolastinc is a prescription medicine used to treat alpha 1 antitrypsin aat deficiency. Alpha1 proteinase inhibitors medical policy pdf blue cross blue. In people who lack the alpha 1antitrypsin protein, breakdown of lung tissues can. Glassia is a liquid medicine containing human alpha 1proteinase inhibitor alpha 1pi also known as alpha 1antitrypsin aat, which is purified from human blood. Alpha 1 proteinase inhibitor injection, also called alpha 1 pi, is used to treat a certain type of emphysema a lung condition. People with alpha1 antitrypsin deficiency usually develop the first signs and symptoms of lung disease between ages 20 and 50. There are no data with alpha1proteinase inhibitor product use in pregnant women to inform a drugassociated risk. Original research the effect of alpha1 proteinase inhibitor.
Alpha1 antitrypsin deficiency genetics home reference nih. An important function of a 1pi in the lung is to inhibit neutrophil elastase, one of various proteolytic enzymes released by activated neutrophils during inflammation. Respreeza 4,000 mg powder and solvent for solution for infusion. Recommended authorization criteria coverage of prolastinc, aralast np, zemaira, or glassia is recommended in those who meet the following criteria. Aralast np, glassia, prolastin, prolastinc, and zemaira alpha1 proteinase inhibitors will be considered for coverage under the pharmacy benefit program when the following criteria are met. Alpha 1 proteinase inhibitor human pubmed central pmc. It is predominantly synthesized in hepatocytes, but is also produced, to a lower extent, by alveolar macrophages, neutrophils. Alpha1proteinase inhibitor prolastinc for the treatment of alpha1. Current specialty pharmacy drugs as of april 1, 2020. Dailymed aralast np alpha1proteinase inhibitor human kit. Prior authorization is recommended for prescription benefit coverage of an alpha 1proteinase inhibitor. Ne is released by activated neutrophils and can degrade elastin within connective tissues. Find information on alpha 1proteinase inhibitor use, treatment, drug class and molecular formula. Review request for alpha 1 proteinase inhibitor therapy.
This protein occurs naturally in the body and is important for preventing the breakdown of tissues in the lungs. A regional health authority has been asked to approve augmentation therapy for a patient with fev1 of less than 30% predicted. Kamada announces extension of glassia alpha1proteinase. Alpha 1proteinase inhibitors aralast np, glassia, prolastinc and zemaira are proven and medically necessary for chronic augmentation and maintenance therapy of patients with emphysema due to congenital deficiency of alpha 1proteinase inhibitor a 1.
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. Alpha 1proteinase inhibitor is used to treat alpha 1antitrypsin deficiency in people who have symptoms of emphysema. Individual is 18 years of age or older treatment of congenital alpha1antitrypsin deficiencyassociated lung disease alpha 1. The main search concepts were prolastinc alpha1proteinase inhibitor human and aat deficiency. Alpha 1proteinase inhibitor may also be used for purposes not listed in this medication guide. A protease inhibitor, it is also known as alpha 1 proteinase inhibitor a1pi or alpha 1antiproteinase a1ap because it. Intravenous therapy with purified plasmaderived alpha1proteinase inhibitor. This may result in shortness of breath, wheezing, or an increased risk of lung infections. Human alpha1 proteinase inhibitor also known as alpha1 antitrypsin is a protein normally present in the body that helps prevent breakdown of tissues in the lungs. Aralast np increases antigenic and functional antineutrophil elastase capacity, anec serum levels and antigenic lung epithelial lining fluid levels of. Glassia alpha1proteinase inhibitor human prolastinc alpha1proteinase inhibitor human zemaira alpha.
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. Individual has a diagnosis of congenital alpha 1 antitrypsin deficiency alpha 1 proteinase inhibitor deficiency. Alpha1proteinase inhibitors are considered investigational and not medically necessary for any indications other than those listed above due to insufficient evidence of therapeutic value. Recent advances in the research and development of alpha1. Alpha1proteinase inhibitors aralast np, glassia, prolastinc, zemaira alpha1proteinase 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 1pi, also known as alpha 1antitrypsin aat deficiency. Respreeza, innhuman alpha1proteinase inhibitor european. Exceptions to these conditions of coverage are considered through the.
Ne is released by activated neutrophils and can degrade elastin within connective. 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. Aralast np is an alpha 1proteinase inhibitor human alpha 1pi indicated for chronic augmentation therapy in adults with clinically evident emphysema due to severe congenital deficiency of alpha 1pi alpha 1antitrypsin deficiency. The case for clinical and costeffectiveness has not been proven. Lung density was measured as adjusted pd15 gl per year. Alpha 1proteinase inhibitor is a protein, also called alpha 1antitrypsin. The total protein content is approximately 1,100 mg per vial. The emphysema is caused by the lack of a protein called alpha 1 antitrypsin aat in the body. Indefinite authorizationmay be granted for members prescribed an alpha. Animal reproduction studies have not been conducted. Alpha1proteinase inhibitors aralast np, glassia, prolastinc. D iagnosis of emphysema due to alpha 1 antitrypsin aat deficiency. Pdf clinical utility of alpha1 proteinase inhibitor in the. Alpha 1 proteinase inhibitor therapy is considered medically appropriate if all of the following criteria are met.
Alpha1 antitrypsin deficiency is an inherited disorder that may cause lung disease and liver disease. May 27, 2019 alpha 1 proteinase inhibitor is used to treat alpha 1 antitrypsin deficiency in people who have symptoms of emphysema. Glassia alpha 1 proteinase inhibitor, human enzyme replacement granix tbofilgrastim blood modifier. Biochemical comparison of four commercially available human. Alpha1 proteinase inhibitor prolastinc is a prescription medicine used to treat alpha1 antitrypsin aat deficiency. The emphysema is caused by the lack of a protein called alpha 1antitrypsin aat in the body. Alpha1proteinase inhibitor products should be given during pregnancy only if clearly needed. Alpha1 antitrypsin deficiency aatd is a rare genetic disease characterized by low serum levels of alpha1 03 proteinase inhibitor a1pi, also known as alpha1 antitrypsin. Efficacy and safety of alpha1proteinase inhibitor human. What is the current clinical evidence to support the use of alpha1 proteinase inhibitor therapy for patients with alpha 1 antitrypsin deficiency and a fev1 less than 30%. Kamada announces extension of glassia alpha1proteinase inhibitor human supply and distribution agreement with takeda through 2021 and expected transition of glassia manufacturing to takeda. Specialty guideline management alpha1proteinase inhibitors. Find patient medical information for alpha1proteinase inhibitor human intravenous on webmd including its uses, side effects and safety, interactions, pictures, warnings and user ratings. In people who lack the alpha 1antitrypsin protein, breakdown of lung tissues can lead to emphysema damage to the air sacs in the lungs.
Over time, significant lung damage occurs, and patients develop serious lung diseases like emphysema. Inflammation of the lungs is one of the causes of chronic lung disease cld in babies born before 37 weeks. And patient has an fev 1 in the range of 3065% of predicted. Prolastinc is an alpha1proteinase inhibitor a1pi available in canada. Find information on alpha 1 proteinase inhibitor use, treatment, drug class and molecular formula. Alpha1proteinase inhibitor injection medication guide note. The patient has an alpha1 proteinase inhibitor alpha1 antitrypsin deficiency and the patient has a diagnosis of emphysema and. The earliest symptoms are shortness of breath following mild. There are no data with alpha 1 proteinase inhibitor product use in pregnant women to inform a drugassociated risk. 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. Intravenous alpha1 proteinase inhibitor will be used as augmentation addition to improve or complete therapy. Alpha 1proteinase inhibitors aralast np, glassia, prolastinc and zemaira are proven and medically necessary for chronic augmentation and maintenance therapy of patients with emphysema due to congenital deficiency of alpha 1proteinase inhibitor a 1pi, also known as alpha 1antitrypsin aat deficiency. Jan 30, 2019 glassia, is an alpha 1 proteinase inhibitor human, indicated for chronic augmentation and maintenance therapy in individuals with clinically evident emphysema due to severe hereditary deficiency of alpha 1 pi, also known as alpha 1 antitrypsin aat deficiency. Medical necessity criteria page 14 alpha1proteinase inhibitors.
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. Food and drug administration fdaapproved indications 1. Efficacy and safety of alpha1proteinase inhibitor human, modified process alpha1 mp in subjects with pulmonary emphysema due to alpha1 antitrypsin deficiency aatd full. Lists the various brand names available for medicines containing alpha 1proteinase inhibitor. Alpha 1antitrypsin deficiency is a genetic inherited disorder and alpha 1proteinase inhibitor will not cure this condition. Alpha 1proteinase inhibitor therapy is considered medically appropriate if all of the following criteria are met. This deficiency is a genetic disorder and cant be cured, but replacement. Prolastinc chronic augmentation and maintenance therapy in adults with clinical evidence of emphysema due to severe hereditary deficiency of alpha1proteinase inhibitor alpha1antitrypsin deficiency 4. May 06, 2019 aralast np is an alpha 1proteinase inhibitor human alpha 1pi indicated for chronic augmentation therapy in adults with clinically evident emphysema due to severe congenital deficiency of alpha 1pi alpha 1antitrypsin deficiency. Alpha 1 proteinase inhibitor products should be given during pregnancy only if clearly needed. Pdf human alpha1proteinase inhibitor is a wellcharacterized protease inhibitor with a wide spectrum of antiprotease activity.
Human alpha 1 proteinase inhibitor also known as alpha 1 antitrypsin is a protein normally present in the body that helps prevent breakdown of tissues in the lungs. Proteases are enzymes which must be carefully regulated, otherwise they can attack and damage normal tissue. Alpha 1 antitrypsin deficiency a1ad or aatd is a genetic disorder that may result in lung disease or liver disease. The all wales medicines strategy group has advised march 2017 that human alpha1 proteinase inhibitor respreeza is not recommended for use within nhs wales for maintenance treatment, to slow the progression of emphysema in adults with documented severe alpha 1proteinase inhibitor deficiency. Intravenous alpha 1 proteinase inhibitor human aralast np, glassia, prolastinc, zemaira is considered medically necessary when all of the following criteria are met. Emphysema due to alpha1antitrypsin aat deficiency patient is 18 years or older. Numbers of patients are 75 for a1pi earlystart group and 64 for placebo delayedstart group.
Human alpha1 proteinase inhibitors prices and information. A deficiency of alpha 1 proteinase inhibitor can lead to emphysema. 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. Above criteria are not met emphysema is due to environmental triggers emphysema is caused by tobacco use. Current specialty pharmacy drugs as of april 1, 2020 brand name new drugs indicated in red ingredient class class cytarabine gytarabine oncology cytogam cytomegalovirus immune globulin intravenous cmvivig immune globulin.
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. Alpha1 antitrypsin deficiency aatd is a common hereditary disorder caused by mutations in the serpina1 gene, which encodes alpha1 antitrypsin aat. The member displays clinically evident emphysema ii. Lists the various brand names available for medicines containing alpha 1 proteinase inhibitor. People with alpha 1 antitrypsin deficiency usually develop the first signs and symptoms of lung disease between ages 20 and 50. Alpha1proteinase inhibitor memorial sloan kettering cancer. Longterm clinical outcomes following treatment with alpha. 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. Individual has a diagnosis of congenital alpha1 antitrypsin deficiency alpha1 proteinase inhibitor deficiency. This product profiler introduces health care professionals to alpha 1proteinase inhibitor human, zemaira, a u. Alpha 1 antitrypsin deficiency is a genetic inherited disorder and alpha 1 proteinase inhibitor will not cure this condition.
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. Glassia alpha1 proteinase inhibitor human prolastinc alpha1 proteinase inhibitor human zemaira alpha. And patient has alpha1antitrypsin aat deficiency with pizz, piz null, or pi null, null phenotypes. There is not enough evidence to show the long term effect of using alpha. Current specialty pharmacy drugs as of april 1, 2020 new drugs indicated in red brand name ingredient class class abilify maintena aripiprazole long acting antipsychotics. The members pretreatment serum aat level is less than 11 m. A deficiency of alpha1 proteinase inhibitor can lead to emphysema. Alpha1proteinase inhibitor memorial sloan kettering. D iagnosis of emphysema due to alpha1antitrypsin aat deficiency. Alpha 1 proteinase inhibitor may also be used for purposes not listed in this medication guide. Onset of lung problems is typically between 20 and 50 years old. Alpha1 proteinase inhibitor injection, also called alpha 1pi, is used to treat a certain type of emphysema a lung condition. Aralast np, glassia, prolastinc, zemaira intravenous.
Bronchiectasis without alpha 1 antitrypsin deficiency. Alpha1 antitrypsin deficiency aatd is a relatively common but underrecognized genetic disorder in which the protease inhibitor, aat, is. Alpha 1proteinase inhibitor human aralasto solvent detergent treated nanofiltered for intravenous use only product information description alpha 1proteinase inhibitor human, aralast. A low concentration of aat is associated with slowly progressive, moderateto. Alpha1 proteinase inhibitors pharmacy coverage policy. Alpha1 proteinase inhibitor therapy for alpha1antitrypsin deficiency 2. Alpha1 antitrypsin deficiency a1ad or aatd is a genetic disorder that may result in lung disease or liver disease.
Deficiency of alpha 1proteinase inhibitor a 1pi, also known as alpha 1 antitrypsin aat deficiency is a chronic, hereditary disorder, characterized by reduced levels of a1pi in the blood and lungs. Alpha1 proteinase inhibitor human intravenous route. If you have an allergy to alpha 1proteinase inhibitor or any other part of this drug. Alpha1proteinase inhibitors aralast np, glassia, prolastinc and. Nov 09, 2018 alpha 1 proteinase inhibitor is a protein, also called alpha 1 antitrypsin. 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. The proteinase inhibitor, alpha1 antitrypsin aat is predominantly produced in the liver1 and helps to regulate proteases2. 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. Studies have not demonstrated alpha 1 proteinase inhibitor to be effective for this condition. Individual has an fev 1 in the range of 3065% of predicted. Bronchiectasis without alpha 1antitrypsin deficiency. The main purpose of infusing glassia is to increase the levels of the aat protein in your blood and lungs.
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