Subject: Alpha1-Proteinase Inhibitors 1877-A SGM P2022

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:
           1.  Pretreatment serum alpha₁-antitrypsin (AAT) level
           2.  Pretreatment post-bronchodilation forced expiratory volume
                in 1 second (FEV₁)
           3.  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:
           1.  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).^5,6

           2.  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.⁶

          3.  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).⁶

          4.  The member does not have the PiMZ or PiMS AAT
               deficiency.⁷

 

    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]. Westlake Village, CA: Baxalta US Inc.; December 2018.
2. Glassia [package insert]. Westlake Village, CA: Baxalta US Inc.; June 2017.
3. Prolastin-C [package insert]. Research Triangle Park, NC: Grifols Therapeutics Inc.; May 2020.
4. Zemaira [package insert]. Kankakee, IL: CSL Behring LLC; April 2019.
5. 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.
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. Can Respir J. 2012;19:109-116.
7. 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 26, 2022