CurrentViva HealthPolicy IC-0052

Alpha-1-Proteinase Inhibitors (Aralast NP, Glassia, Prolastin-C, Zemaira)

Defines prior authorization, clinical criteria, dosing, renewal, quantity limits, billing codes/NDCs, and covered diagnoses for intravenous alpha-1-proteinase inhibitors for emphysema due to alpha-1-antitrypsin deficiency and for steroid-refractory acute graft-versus-host disease (aGVHD).

Policy Summary

PayerViva Health

PolicyAlpha-1-Proteinase Inhibitors (Aralast NP, Glassia, Prolastin-C, Zemaira)

Policy CodePolicy IC-0052

Change TypeNo material change

Effective DateJan 1, 2012

Next Review Date

Key ActionPrior authorization is required; submit documentation of phenotype, FEV1, serum AAT with assay method, smoking status, and prerequisite therapy (failure/intolerance to Glassia® AND Zemaira®).

POLICY UPDATE CHANGES

No material clinical or coverage changes in this update.

2Covered Indications

12 moInitial Authorization Length

2800Max units (emphysema)

8Max doses (aGVHD)

Coverage Summary

This policy covers intravenous alpha-1-proteinase inhibitors (Aralast NP, Glassia, Prolastin-C, Zemaira) for the FDA-approved indication of emphysema due to alpha-1-antitrypsin (AAT) deficiency and for compendia-recommended use in steroid-refractory acute graft-versus-host disease (aGVHD). Covered indications and required clinical criteria (e.g., AAT phenotype, FEV1 thresholds, smoking status, and serum AAT thresholds) are defined in the policy and informed by product labeling and society guidance.

Key limits and requirements include: initial prior authorization is provided for 12 months (365 days) for emphysema (aGVHD initial authorization is limited to up to 8 doses / 4 weeks and may not be renewed); dosing must follow policy (60 mg/kg IV weekly for emphysema; 60 mg/kg IV on days 1,4,8,12,16,20,24,28 up to 8 doses for aGVHD); and billing uses specified HCPCS/NDCs with unit and maximum limits (see billing and max units). The policy references product package inserts and relevant society guidelines for clinical and safety information.

Medical Necessity — Initial and Renewal Criteria

Initial Approval Criteria

Prior authorization validity is provided when ALL of the following are met:

Initial Approval Criteria - top level

Universal Criteria
- Prerequisite therapy: Patient must have a contraindication, intolerance, or failure to Glassia® AND Zemaira® prior to the consideration of another alpha-1 proteinase inhibitor product
- Age: Member is at least 18 years of age≥ 18 years
- FDA labeled contraindications: Member does NOT have any FDA labeled contraindications to the requested agent
Indication-specific (choose one indication and meet its criteria)
- Emphysema due to alpha-1-antitrypsin (AAT) deficiency
  - FEV1: Member has an FEV1 in the range of 30-65% of predicted30-65%
  - Phenotype: Member has alpha-1-antitrypsin (AAT) deficiency with PiZZ, PiZ (null), or Pi (null, null) phenotypesPiZZ, PiZ (null), or Pi (null,null)
  - Emphysema pattern: Member has clinical evidence of panacinar/panlobular emphysema or centrilobular emphysema
  - Serum AAT concentration:

Renewal Criteria

Prior authorization may be renewed when ALL of the following are met:

Renewal Criteria - top level

Continuation of initial criteria: Member continues to meet the universal and indication-specific relevant criteria identified in Initial Approval Criteria (section III)
Duration of authorization has not been exceeded (refer to Length of Authorization)Initial: 12 months (365 days); aGVHD: up to 8 doses (4 weeks) and may NOT be renewed
Toxicity: Absence of unacceptable toxicity from the drug (examples include severe hypersensitivity reactions)
Emphysema-specific evidence of benefit (any one suffices):

Coding

HCPCS codes for alpha-1 proteinase inhibitorsHCPCSCovered

J0256	Aralast NP, Prolastin-C, Zemaira (1 billable unit = 10 mg)
J0257	Glassia (1 billable unit = 10 mg)

NDCs (listed product presentations)NDC

00944-2815-xx	Aralast NP 1 g/50 mL
00944-2814-xx	Aralast NP 0.5 g/25 mL
00944-2884-xx	Glassia 1 g/50 mL
13533-0705-xx	Prolastin-C Liquid (multiple SDV sizes: 500 mg/10 mL; 1 g/20 mL; 4 g/80 mL)
00053-7201-xx	Zemaira 1 g/20 mL
00053-7202-xx	Zemaira 4 g/76 mL
00053-7203-xx	Zemaira 5 g/95 mL

Covered Diagnosis Codes (ICD-10)ICD-10Covered

D89.810	Acute graft-versus-host disease
D89.812	Acute on chronic graft-versus-host disease
D89.813	Graft-versus-host disease, unspecified
E88.01	Alpha-1-antitrypsin deficiency
T86.09	Other complications of bone marrow transplant

Billing unit conversion: 1 billable unit = 10 mg. Dosing and billing must follow the policy dosing schedules and HCPCS codes (J0256 or J0257) with appropriate NDC/SDV selection and adherence to the policy's maximum billable unit limits; see the policy billing rule for full billing and unit calculation details.

Provider Actions & Requirements

Prior Authorization

Prior authorization required

Prior authorization is required.

Initial authorization length: 12 months (365 days).
Exception — Acute graft-versus-host disease (aGVHD): authorized for up to 8 doses (4 weeks) and may NOT be renewed.

Documentation Required

Prerequisite therapy documentation

Document that the member has a contraindication, intolerance, or failure to Glassia® AND Zemaira® before approving another alpha‑1 product. Also document the following clinical elements.

Phenotype: PiZZ, PiZ (null), or Pi (null, null).
FEV1 percent predicted.
Serum AAT level with assay method specified (nephelometry or ELISA).
Smoking status.
Evidence of optimized medical therapy (e.g., bronchodilators, inhaled corticosteroids, pulmonary rehabilitation, vaccinations, smoking cessation, supplemental oxygen, case management).
Affected diagnosis codes: E88.01, D89.810, D89.812, D89.813, T86.09

Billing Rule

Dose and billing unit adherence

Dose and billing must follow policy-specified regimens and HCPCS unit reporting.

Emphysema due to AAT deficiency: 60 mg/kg IV once weekly.
Acute GVHD: 60 mg/kg IV on days 1, 4, 8, 12, 16, 20, 24, and 28 (up to 8 doses / 4 weeks).
Billing: use HCPCS J0256 or J0257; 1 billable unit = 10 mg.
Max units: emphysema — 2800 units every 28 days; aGVHD dosing period — 700 units per dose (total up to 8 doses = 5600 units per 28 days).
Include appropriate NDC/Single‑Dose Vial (SDV) information when required.

Denial Risk

Denial risk for smoking or inadequate phenotype/levels

Claims may be denied if required clinical criteria are not met.

Active tobacco smoker — denial risk.
Lack of required phenotype (must be PiZZ, PiZ (null), or Pi (null, null)).
Serum AAT above policy thresholds — denial if not ≤ 57 mg/dL (nephelometry) or not ≤ 11 µM/L (ELISA).
FEV1 outside required range — must be 30–65% of predicted.

Background & Evidence

Covered products include Aralast NP, Glassia, Prolastin-C, and Zemaira (intravenous alpha-1-proteinase inhibitors). The FDA-approved indication is augmentation therapy for emphysema due to AAT deficiency; the policy also covers compendia-recommended use for steroid-refractory acute GVHD as an additional therapy in combination with systemic corticosteroids following failure of first-line options. Product labeling (package inserts) and society guidelines (e.g., ATS/ERS, ERS, NCCN compendia) are referenced throughout the policy for clinical details, dosing, contraindications, and safety information.

Evidence sources cited in the policy include package inserts for Glassia, Zemaira, Aralast NP, and Prolastin‑C; statements and guidance from respiratory societies such as the American Thoracic Society/European Respiratory Society (ATS/ERS) and European Respiratory Society (ERS); the NCCN Drugs & Biologics Compendium; and clinical studies and guideline publications from 2024–2026 where applicable.