Subject: Alpha1-Proteinase Inhibitors 1877-A SGM P2024

Drug

Alpha1-Proteinase Inhibitors

ARALAST NP  (alpha1-proteinase inhibitor [human])

GLASSIA  (alpha1-proteinase inhibitor [human])

PROLASTIN-C  (alpha1-proteinase inhibitor [human])

ZEMAIRA  (alpha1-proteinase inhibitor [human])

Policy:

      I.  INDICATIONS

          The indications below including FDA-approved indications and compendial uses are
          considered a covered benefit provided that all the approval criteria are met and the member
          has no exclusions to the prescribed therapy.

          FDA-Approved Indications
          1.  Aralast NP
               Chronic augmentation therapy in adults with clinically evident emphysema due to severe
               congenital deficiency of alpha₁-proteinase inhibitor (alpha₁-antitrypsin deficiency)

          2.  Glassia
               Chronic augmentation and maintenance therapy in adults with clinically evident emphysema
               due to severe hereditary deficiency of alpha₁-proteinase inhibitor (alpha₁-antitrypsin
               deficiency)

          3.  Prolastin-C
               Chronic augmentation and maintenance therapy in adults with clinical evidence of
               emphysema due to severe hereditary deficiency of alpha₁-proteinase inhibitor (alpha₁-
               antitrypsin deficiency)

          4.  Zemaira
               Chronic augmentation and maintenance therapy in adults with alpha₁-proteinase inhibitor
               deficiency and clinical evidence of emphysema

          All other indications are considered experimental/investigational and not medically necessary.


    II.  DOCUMENTATION

          Submission of the following information is necessary to initiate the prior authorization review:
          A.  Pretreatment serum alpha₁-antitrypsin (AAT) level
          B.  Pretreatment post-bronchodilation forced expiratory volume in 1 second (FEV₁)
          C.  AAT protein phenotype or genotype


   III.  CRITERIA FOR INITIAL APPROVAL

          Authorization of 12 months may be granted for treatment of emphysema due to alpha₁-
          antitrypsin (AAT) deficiency when all of the following criteria are met:
          A.  The member’s pretreatment serum AAT level is less than 11 micromol/L (80 mg/dL by radial
               immunodiffusion or 50 mg/dL by nephelometry).
          B.  The member’s pretreatment post-bronchodilation forced expiratory volume in 1 second
               (FEV₁) is greater than or equal to 25% and less than or equal to 80% of the predicted value.
          C.  The member has a documented PiZZ, PiZ (null), or Pi (null, null) (homozygous) AAT
               deficiency or other phenotype or genotype associated with serum AAT concentrations of
               less than 11 micromol/L (80 mg/dL by radial immunodiffusion or 50 mg/dL by nephelometry).
          D.  The member does not have the PiMZ or PiMS AAT deficiency.


   IV.  CONTINUATION OF THERAPY

          Authorization of 12 months may be granted for continued treatment of emphysema due to
          alpha₁-antitrypsin (AAT) deficiency when the member is experiencing beneficial clinical
          response from therapy.


    V.  OTHER

          Note: If the member is a current smoker, they should be counseled on the harmful effects of
          smoking on pulmonary conditions and available smoking cessation options.

 

Place of Service:

Outpatient

The above policy is based on the following references:

1. Aralast NP [package insert]. Lexington, MA: Baxalta US Inc.; March 2023.
2. Glassia [package insert]. Lexington, MA: Takeda Pharmaceuticals US Inc.; September 2023.
3. Prolastin-C Liquid [package insert]. Research Triangle Park, NC: Grifols Therapeutics LLC.; May 2020.
4. Prolastin-C [package insert]. Research Triangle Park, NC: Grifols Therapeutics LLC.; January 2022.
5. Zemaira [package insert]. Kankakee, IL: CSL Behring LLC; September 2022.
6. American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med. 2003;168:818-900.
7. Marciniuk DD, Hernandez P, Balter M, et al. Alpha-1 antitrypsin deficiency targeted testing and augmentation therapy: a Canadian Thoracic Society clinical practice guideline. Can Respir J. 2012;19:109-116.
8. 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):668-82.

 

Copyright Aetna Inc. All rights reserved. Pharmacy Clinical Policy Bulletins are developed by Aetna to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. This Clinical Policy Bulletin contains only a partial, general description of plan or program benefits and does not constitute a contract. Aetna does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Aetna or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. This Clinical Policy Bulletin may be updated and therefore is subject to change.

June 16, 2024