Alpha-1 Proteinase Inhibitors

Indications for Prior Authorization

Aralast NP (alpha-1-proteinase inhibitor [human])
  • For diagnosis of Alpha-1 proteinase inhibitor deficiency (also known as alpha-1-antitrypsin (AAT) deficiency)
    Indicated for chronic augmentation therapy in adults with clinically evident emphysema due to severe congenital deficiency of Alpha1-PI (alpha1-antitrypsin deficiency). Aralast NP increases antigenic and functional (anti-neutrophil elastase capacity, ANEC) serum levels and antigenic lung epithelial lining fluid levels of Alpha1-PI. The effect of augmentation therapy with Alpha1-PI, including Aralast NP, on pulmonary exacerbations and on the progression of emphysema in alpha-1-antitrypsin deficiency has not been conclusively demonstrated in randomized, controlled clinical trials. Clinical data demonstrating the long-term effects of chronic augmentation and maintenance therapy with Aralast NP or Aralast are not available. Aralast NP is not indicated as therapy for lung disease patients in whom severe congenital Alpha-1-PI deficiency has not been established.

Glassia (alpha-1-proteinase inhibitor [human])
  • For diagnosis of Alpha-1 proteinase inhibitor deficiency (also known as alpha-1-antitrypsin (AAT) deficiency)
    Indicated for chronic augmentation and maintenance therapy in individuals with clinically evident emphysema due to severe hereditary deficiency of Alpha1-PI, also known as alpha1-antitrypsin (AAT) deficiency.

    Limitations of Use: The effect of augmentation therapy with Glassia or any Alpha1-PI product on pulmonary exacerbations and on the progression of emphysema in Alpha1-PI deficiency has not been conclusively demonstrated in randomized, controlled clinical trials. Clinical data demonstrating the long-term effects of chronic augmentation and maintenance therapy of individuals with Glassia are not available. Glassia is not indicated as therapy for lung disease in patients in whom severe Alpha1-PI deficiency has not been established.

Prolastin-C (alpha-1-proteinase inhibitor [human]), Prolastin-C liquid (alpha-1-proteinase inhibitor [human])
  • For diagnosis of Alpha-1 proteinase inhibitor deficiency (also known as alpha-1-antitrypsin (AAT) deficiency)
    Indicated for chronic augmentation and maintenance therapy in adults with clinical evidence of emphysema due to severe hereditary deficiency of Alpha1-PI (alpha1-antitrypsin deficiency). Prolastin-C increases antigenic and functional (anti-neutrophil elastase capacity, ANEC) serum levels and antigenic lung epithelial lining fluid levels of Alpha1-PI. Limitations of Use: The effect of augmentation therapy with any Alpha-1-PI product on pulmonary exacerbations and on the progression of emphysema in Alpha1-PI deficiency has not been conclusively demonstrated in randomized, controlled clinical trials. Clinical data demonstrating the long-term effects of chronic augmentation or maintenance therapy with Prolastin-C are not available. Prolastin-C is not indicated as therapy for lung disease in patients in whom severe Alpha-1-PI deficiency has not been established.

Zemaira (alpha-1-proteinase inhibitor [human])
  • For diagnosis of Alpha-1 proteinase inhibitor deficiency (also known as alpha-1-antitrypsin (AAT) deficiency)
    Indicated for chronic augmentation and maintenance therapy in adults with Alpha1-PI deficiency and clinical evidence of emphysema. Zemaira increases antigenic and functional (ANEC) serum levels and lung epithelial lining fluid levels of Alpha1-PI. Clinical data demonstrating the long-term effects of chronic augmentation therapy of individuals with Zemaira are not available. The effect of augmentation therapy with Zemaira or any Alpha1-PI product on pulmonary exacerbations and on the progression of emphysema in Alpha1-PI deficiency has not been demonstrated in randomized, controlled clinical trials. Zemaira is not indicated as therapy for lung disease patients in whom severe Alpha1-PI deficiency has not been established.

Criteria

Aralast NP, Glassia, Prolastin-C, Prolastin-C liquid, or Zemaira

Prior Authorization (Initial Authorization)

Length of Approval: 12 Month(s)

  • Diagnosis of congenital alpha-1 antitrypsin (AAT) deficiency
  • AND
  • Diagnosis of emphysema [A]
  • AND
  • One of the following:
    • Pi*ZZ, Pi*Z(null) or Pi*(null)(null) protein phenotypes (homozygous) [6]
    • OR
    • Other rare AAT disease genotypes associated with pre-treatment serum alpha1-antitrypsin (AAT) level less than 11 micromole per liter [e.g., Pi(Malton, Malton), Pi(SZ)] [B]
    AND
  • One of the following:
    • Circulating pre-treatment serum alpha1-antitrypsin (AAT) level less than 11 micromole per liter (which corresponds to less than 80 mg/dL if measured by radial immunodiffusion or less than 57 mg/dL if measured by nephelometry) [B, 10]
    • OR
    • Patient has a concomitant diagnosis of necrotizing panniculitis
    AND
  • Continued optimal conventional treatment for emphysema (e.g., bronchodilators)
  • AND
  • One of the following: [8, 9, 10]
    • The FEV1 level is less than or equal to 65% of predicted
    • OR
    • Patient has experienced a rapid decline in lung function (i.e., reduction of FEV1 more than 120 mL/year) that warrants treatment [9]
    • OR
    • Patient has a concomitant diagnosis of necrotizing panniculitis
    AND
  • Patient is NOT a current smoker [C]
Aralast NP, Glassia, Prolastin-C, Prolastin-C liquid, or Zemaira

Prior Authorization (Reauthorization)

Length of Approval: 12 Month(s)

  • Patient demonstrates positive clinical response to therapy
  • AND
  • Continued optimal conventional treatment for emphysema (e.g., bronchodilators)
P & T Revisions

2024-02-05, 2023-09-29, 2023-02-22, 2022-08-04, 2022-02-23, 2021-09-27, 2021-05-18, 2021-02-19, 2020-12-24, 2020-03-12

  1. Aralast NP Prescribing Information. Baxalta US Inc. Westlake Village, CA. March 2023.
  2. Zemaira Prescribing Information. CSL Behring LLC. Kankakee, IL. September 2022.
  3. American Thoracic Society/European Respiratory Society Statement: Standards for diagnosis and management of individuals with alpha-1 antitrypsin deficiency. Am J Resp Care Med 2003; 168:818-900.
  4. Prolastin-C Prescribing Information. Grifols Therapeutics, Inc. Research Triangle Park, NC. January 2022.
  5. Glassia Prescribing Information. Baxalta US Inc. Lexington, MA. September 2023.
  6. Marciniuk DD, Hernandez P, Balter M, et al. Alpha-1 antitrypsin deficiency targeted testing and augmentation therapy: A Canadian Thoracic Society clinical practice guideline. Canadian Respiratory Journal 2012;19(2):109-116.
  7. Stoller JK. Treatment of of alpha-1 antitrypsin deficiency. UpToDate. Accessed March 12, 2019.
  8. Vogelmeir C, Agusti A, et al. The global strategy for diagnosis, management and prevention of COPD (2020 Report). Global Initiative for Chronic Obstructive Lung Disease. Accessed January 21, 2020.
  9. Brantly ML, Lascano JE, Shahmohammadi A. Intravenous alpha-1 antitrypsin therapy for alpha-1 antitrypsin deficiency: the current state of the evidence. Chronic Obstr Pulm Dis. 2019;6(1):100-114.
  10. Sandhaus RA, Turino G, Brantly ML, et al. The diagnosis and management of alpha-1 antitrypsin deficiency in the adult. Chronic Obstr Pulm Dis. 2016; 3(3): 668-682.

  1. Currently, augmentation therapy is not recommended for patients without emphysema. [3, 8] Some individuals with AAT deficiency will not go on to develop panacinar emphysema, only those with evidence of such disease should be considered for augmentation therapy.
  2. Population studies suggest a minimum plasma threshold of 11 μmol/L (corresponding to 80 mg/dL in some assays and ~57 mg/dL by nephelometry), below which there is insufficient AAT to protect the lung, leading to a risk of developing emphysema. [3, 6-9]
  3. The GOLD report recommends reserving alpha-1 antitrypsin augmentation therapy for those with evidence of continued and rapid progression following smoking cessation. [8]

  • 2024-02-05: 2024 UM Annual Review. No criteria changes. Background updates
  • 2023-09-29: Program update to standard reauthorization language. No changes to clinical intent.
  • 2023-02-22: 2023 UM Annual Review. No changes to criteria. Updated references
  • 2022-08-04: Minor formatting update
  • 2022-02-23: 2022 UM Annual Review.
  • 2021-09-27: Addition of EHB formulary to guideline, no changes to criteria
  • 2021-05-18: Addition of EHB formulary to guideline, no changes to criteria
  • 2021-02-19: 2021 UM Annual Review.
  • 2020-12-24: Updated criteria to align with Stage 5 CMS Review concern to allow an exception for diagnosis of concomitant necrotizing panniculitis.
  • 2020-03-12: 2020 UM Annual Review. No changes to criteria.

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