CurrentBlue Cross Blue Shield - IowaPolicy 05.02.39

Alpha1-Proteinase Inhibitors (alpha1-antitrypsin) coverage

Criteria for coverage of alpha1-proteinase inhibitor products (Prolastin-C, Zemaira, Aralast NP, Glassia) for augmentation/maintenance therapy in adults with emphysema due to alpha1-antitrypsin (AAT) deficiency and compendial use for steroid-refractory acute graft-versus-host disease (GVHD); applies to Wellmark members where benefits exist.

Policy Summary

PayerBlue Cross Blue Shield - Iowa

PolicyAlpha1-Proteinase Inhibitors (alpha1-antitrypsin) coverage

Policy CodePolicy 05.02.39

Change TypeNo material changes

Effective DateJune 8, 2025

Next Review DateN/A

Key ActionSubmit prior authorization with pretreatment serum AAT level, AAT phenotype/genotype, and post-bronchodilation FEV1; approvals are typically granted for 12 months and targeted products require prior use and intolerance to preferred products.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12Standard authorization duration for initial and continuation approvals

2Preferred products required before targeted products (Prolastin-C, Zemaira)

25-80Permissible pretreatment post-bronchodilation FEV1 % predicted for initiation

<11Serum AAT threshold for eligibility (micromol/L)

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Authorization Criteria and Duration

Initial Approval — Alpha1-Proteinase Inhibitor for AAT deficiency (emphysema)

Authorization of 12 months may be granted when ALL of the following are met:

ALL of the following

Pretreatment serum AAT level is less than 11 micromol/L (80 mg/dL by radial immunodiffusion or 50 mg/dL by nephelometry).
Documented AAT genotype/phenotype consistent with severe deficiency (examples: PiZZ, PiZ(null), Pi(null,null), or other phenotype associated with serum AAT < 11 micromol/L).
Pretreatment post-bronchodilation forced expiratory volume in 1 second (FEV1) is ≥ 25% and ≤ 80% of predicted.
Exclude these genotypes
- Member does NOT have PiMZ AAT deficiency.
- Member does NOT have PiMS AAT deficiency.
If requesting a targeted product (Aralast NP or Glassia), the member has experienced an intolerable adverse event with both preferred products Prolastin-C and Zemaira, unless clinical circumstances preclude use of preferred products.

Initial Approval — Acute Graft-Versus-Host Disease (GVHD)

Authorization of 12 months may be granted when ALL listed criteria for this indication are met:

ALL of the following

Indication is steroid-refractory acute GVHD following hematopoietic stem cell transplant.

Continuation of Therapy — AAT deficiency

Authorization of 12 months may be granted for continued treatment when ALL of the following are met:

ALL of the following

Member is experiencing a beneficial clinical response from therapy.

Continuation of Therapy — Acute GVHD

Members requesting continuation must meet all requirements in the coverage criteria for the indication.

ALL of the following

Continuation requests must meet the same coverage criteria as initial approval for steroid-refractory acute GVHD.

Numeric and Clinical Thresholds for Eligibility

Eligibility Thresholds

Serum alpha1-antitrypsin (AAT) level< 11 micromol/L (80 mg/dL by radial immunodiffusion or 50 mg/dL by nephelometry)

Pretreatment post-bronchodilation FEV1>= 25% and <= 80% of predicted

What Providers Must Do

Prior Authorization

Authorization requirement

Prior authorization is required for non-preferred alpha1‑proteinase inhibitor products; approvals are typically for 12 months. Submit required documentation to initiate review. Affected HCPCS J‑codes: J0256, J0257.

J0256
J0257

Documentation Required

Required pretreatment documentation

Providers must submit the following pretreatment documents to initiate the authorization review.

Pretreatment serum alpha1‑antitrypsin (AAT) level
AAT protein phenotype or genotype
Pretreatment post‑bronchodilation FEV1

Billing Codes

HCPCS J-codes (product-specific billing)HCPCS

J0256	Injection, alpha1 proteinase inhibitor (human); not otherwise specified, 10 mg (Prolastin-C, Aralast NP, Zemaira)
J0257	Injection, alpha1 proteinase inhibitor (human), 10 mg (Glassia)

Defined Terms

Definitions

Augmentation therapyReplacement therapy with alpha1-proteinase inhibitor (human) to raise serum AAT levels and potentially slow progression of emphysema in AAT deficiency.

Steroid-refractory acute GVHDAcute graft-versus-host disease that does not respond to standard systemic corticosteroid therapy following hematopoietic stem cell transplant.

Clinical and Administrative Background

This policy addresses augmentation (replacement) therapy with plasma‑derived alpha1‑proteinase inhibitor (alpha1‑antitrypsin, AAT) for adults with clinically evident emphysema due to severe congenital AAT deficiency. FDA‑approved products in this class are plasma‑derived human AAT preparations and are billed with product‑specific HCPCS codes (for example, J0256 and J0257). The program is designed to ensure appropriate patient selection while steering utilization to the plan’s preferred products, with Prolastin‑C and Zemaira identified as the preferred agents and Aralast NP and Glassia designated as targeted (non‑preferred) products. (See product table and billing codes.)

Use of targeted products is limited: requests for Aralast NP or Glassia require prior documentation that the member has experienced an intolerable adverse event with both preferred products, Prolastin‑C and Zemaira, unless clinical circumstances exclude use of the preferred products. Required pretreatment documentation to initiate authorization includes the serum AAT level, AAT protein phenotype or genotype, and pretreatment post‑bronchodilation FEV1. Approvals are typically granted for 12 months when criteria are met.

In addition to augmentation therapy for AAT deficiency, compendial use of alpha1‑proteinase inhibitor products is recognized for steroid‑refractory acute graft‑versus‑host disease (GVHD) following hematopoietic stem cell transplant; continuation requests for GVHD must meet the same coverage criteria as initial approval. The policy also calls for counseling current smokers about the harmful effects of smoking and available cessation options.