Skip to main content
HealthPartners
Site Search

Alpha-1 antitrypsin (AAT) deficiency enzyme replacement therapy: alpha-1 proteinase inhibitor (Aralast/NP, Glassia®, Prolastin/C and Zemaira®)

These services may or may not be covered by your HealthPartners plan. Please see your plan documents for your specific coverage information. If there is a difference between this general information and your plan documents, your plan will be used to determine your coverage.

Administrative process

Drug therapies to replace alpha 1-proteinase inhibitor in individuals with alpha-1 antitrypsin deficiency are not covered without prior authorization from HealthPartners Pharmacy Services.

Coverage for drug therapy in alpha-1 antitrypsin deficiency is generally provided when medically necessary.

Alpha-1 antitrypsin (AAT) inhibitor therapy is medically necessary for members with emphysema due to AAT deficiency when all the following criteria are met:

  1. Low serum concentration of AAT < 11 micromoles/L (or less than 80mg/dL or less than 0.8 g/L), which is considered the threshold thought to protect against emphysema; and
  2. Progressive panacinar emphysema is present with a documented rate of decline in forced expiratory volume in 1 second (FEV1); and
  3. Ex-smoker or non-smoker.

Alpha-1 Antitrypsin Deficiency: Alpha-1 antitrypsin is an antiprotease found in human plasma that inhibits the neutrophil elastase enzyme from degrading elastin tissues in the lung. Deficiency is an inherited disorder associated with the early onset of pulmonary emphysema in adults. Although replacement therapies have not been shown to prevent or reverse emphysema in AAT deficiency, population studies suggest a minimum plasma threshold of 11 micromoles/L, below which there is insufficient AAT to protect the lung leading to emphysema.

Alpha-1 proteinase inhibitor therapy is indicated for chronic augmentation and maintenance therapy in adults with emphysema due to congenital deficiency of alpha1-proteinase inhibitor. The effect of augmentation therapy on pulmonary exacerbations and on the progression of emphysema in alpha1-deficiency has not been demonstrated in randomized, controlled clinical trials. Clinical data demonstrating the long-term effects of chronic augmentation and maintenance therapy of individuals is not available. It is not indicated as therapy for lung disease in patients in whom severe alpha1-proteinase deficiency has not been established.

Currently available alpha-1 proteinase inhibitor products include Aralast NP, Glassia, Prolastin C and Zemaira.

If available, codes are listed below for informational purposes only, and do not guarantee member coverage or provider reimbursement. The list may not be all-inclusive.

HCPCS Codes

J0257 – Injection, alpha-1 proteinase inhibitor (human), (Glassia), 10 mg
J0256 – Injection, alpha-1 proteinase inhibitor, human, 10 mg, not otherwise specified.
S9346 - Home infusion therapy, alpha-1-proteinase inhibitor (e.g., Prolastin); administrative services,
professional pharmacy services, care coordination and all necessary supplies and equipment
(drugs and nursing visits coded separately), per diem

NDC Codes

0944280201 - 400 mg, Aralast NP single use vial containing the labeled amount of functionally active
alpha 1-proteinase inhibitor as stated on the label
0944280202 - 800mg, Aralast NP single use vial containing the labeled amount of functionally active
alpha 1-proteinase inhibitor as stated on the label
0944281201 - 500mg, Aralast NP single use vial containing the labeled amount of functionally active
alpha 1-proteinase inhibitor as stated on the label
0944282202 - 1000mg, Aralast NP single use vial containing the labeled amount of functionally active
alpha 1-proteinase inhibitor as stated on the label
0944288401 - 1000mg, Glassia single use vial containing 1 gram of functional alpha 1-proteinase
inhibitor in 50 mL of solution and a sterile filter needle
13533060130 - 500mg, Prolastin-C single use vial containing the labeled amount of functionally
active alpha 1-proteinase inhibitor, in milligrams, as stated on the label, one 20 mL vial of
Sterile Water for Injection, USP, a transfer needle, and a filter needle
13533060135 - 1000mg, Prolastin-C single use vial containing the labeled amount of functionally
active alpha 1-proteinase inhibitor, in milligrams, as stated on the label, one 20 mL vial of
Sterile Water for Injection, USP, a transfer needle, and a filter needle
13533070001 - 1000mg, Prolastin-C single use vial containing the labeled amount of functionally
active alpha 1-proteinase inhibitor, in milligrams, as stated on the label, one 20 mL vial of
Sterile Water for Injection, USP, a transfer needle, and a filter needle
00053720101 - Zemaira single use vial containing the labeled amount of functionally active
alpha 1-proteinase inhibitor as stated on the label
00053720102 - Carton (kit) containing one vial of Zemaira [NDC 0053721101], one 20 mL vial
of Sterile Water for Injection, USP (diluent) [NDC 0053765320] and one vented transfer
device
00944288401 - 1000mg, Glassia single use vial containing 1 gm of functional alpha 1-proteinase
inhibitor in 50 mL of solution and sterile filter needle

CPT Copyright American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association.

References

  1. Prolastin/C Prescribing Information. www.prolastin.com (Accessed 11/11/10)
  2. Aralast/NP and Glassia Prescribing Information. www.alpha1health.com (Accessed 11/11/10)
  3. Zemaira Prescribing Information. http://www.zemaira.com (Accessed 11/11/10)
  4. Glassia FDA Review. www.fda.gov/BiologicsBloodVaccines/BloodBloodProducts/ApprovedProducts/LicensedProductsBLAs/FractionatedPlasmaProducts/ucm217877.htm (Accessed 10/11/10)
  5. Gotzsche PC, Johansen HK. Intravenous alpha-1 antitrypsin augmentation therapy for treating patients with alpha-1 antitrypsin deficiency and lung disease (Review). The Cochrane Collaboration.2010:7. www.thecochranelibrary.com (Accessed 11/11/10).
  6. American Thoracic Society/European Respiratory Soceity Standards document for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med 2003;168:818.
  7. Abboud RT, Ford GT, and Chapman KR. Alpha1-antitrypsin deficiency: a position statement of the Canadian Thoracic Society. Can Respir J 2001 Mar-Apr;8(2):81-8.

Products

This information is for most, but not all, HealthPartners plans. Please read your plan documents to see if your plan has limits or will not cover some items. If there is a difference between this general information and your plan documents, your plan documents will be used to determine your coverage. These coverage criteria may not apply to Medicare Products if Medicare requires different coverage. For more information regarding Medicare coverage criteria or for a copy of a Medicare coverage policy, contact Member Services at 952-883-7979 or 1-800-233-9645.