Subject: Alpha1-Proteinase Inhibitors 1877-A SGM P2023

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).
          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.
          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).
          4.  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.; December 2018.
2. Glassia [package insert]. Lexington, MA: Takeda Pharmaceuticals US Inc.; March 2022.
3. Prolastin-C Liquid [package insert]. Research Triangle Park, NC: Grifols Therapeutics Inc.; May 2020.
4. Prolastin-C [package insert]. Research Triangle Park, NC: Grifols Therapeutics Inc.; 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 18, 2023