Subject: Alpha1-Proteinase Inhibitors 1877-A SGM P2021

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 

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) phenotype (homozygous) AAT deficiency
     or other phenotype 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 phenotype 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]. 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.; August 2018.
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 14, 2021