CurrentViva HealthPolicy IC-0052

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

Clinical coverage and prior authorization criteria for intravenous alpha-1-proteinase inhibitors for treatment of emphysema due to alpha-1-antitrypsin deficiency and for compendia-recommended use in steroid-refractory acute graft-versus-host disease (aGVHD). Applies to Viva Health medical benefit drug coverage.

Policy Summary

PayerViva Health

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

Policy CodePolicy IC-0052

Change TypeNo material changes

Effective DateJan 1, 2012

Next Review Date

Key ActionObtain prior authorization and document prerequisite therapy, AAT phenotype and serum level, and required clinical measures for initial and renewal requests.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12Initial prior authorization duration (standard)

8Maximum prior authorization for aGVHD

60 mg/kgRecommended dosing for AAT deficiency

30-65%Required FEV1 range for emphysema indication

Medical Necessity Criteria

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Initial Authorization — General / Universal Criteria

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

ALL of the following

Patient has no FDA labeled contraindications to the requested alpha-1-proteinase inhibitor
Member is at least 18 years of age
Patient has a contraindication, intolerance, or failure to Glassia AND Zemaira prior to consideration of another alpha-1 proteinase inhibitor product
This prerequisite applies prior to coverage of an alternative alpha-1 product
Member is receiving optimal medical therapy (examples: bronchodilators, inhaled corticosteroids, comprehensive case management, pulmonary rehabilitation, vaccinations, smoking cessation, supplemental oxygen, self-management skills, etc.)

Initial Authorization — Emphysema due to AAT deficiency (FDA indication)

In addition to Universal Criteria, ALL of the following must be met for coverage of alpha-1-proteinase inhibitor for emphysema due to AAT deficiency:

ALL of the following

FEV1 is in the range of 30-65% of predicted30-65% predicted
Alpha-1-antitrypsin (AAT) deficiency with phenotype PiZZ, PiZ (null), or Pi (null, null)
Clinical evidence of panacinar/panlobular emphysema or centrilobular emphysema
Low serum concentration of AAT ≤ 57 mg/dL (nephelometry) OR ≤ 11 µM/L (ELISA)≤ 57 mg/dL OR ≤ 11 µM/L
Member is not a tobacco smoker

Initial Authorization — Acute Graft Versus Host Disease (aGVHD) (Compendia recommended)

For compendia-recommended use in steroid-refractory aGVHD, ALL of the following must be met:

ALL of the following

Member has received a hematopoietic stem cell transplant
Used for steroid-refractory acute GVHD
Used in combination with systemic corticosteroids as additional therapy following no response to first-line therapy options

Renewal Criteria

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

ALL of the following

Member continues to meet the universal and indication-specific criteria (excluding prerequisite therapy), including concomitant therapy requirements and performance status
Duration of authorization has not been exceeded (see Length of Authorization section)
Absence of unacceptable toxicity from the drug (examples include severe hypersensitivity reactions)

For emphysema due to AAT deficiency, at least ONE of the following disease response measures is required

Elevation of AAT levels above baseline

What Providers Must Do

Prior Authorization

Obtain prior authorization (use J0256 or J0257)

Prior authorization is required for requests for alpha-1-proteinase inhibitors. Initial authorizations are typically valid for 12 months (365 days). For acute GVHD (aGVHD) requests, authorization is provided for a maximum of 8 doses (4 weeks) and is not renewable. Submit requests using HCPCS codes J0256 or J0257 as applicable (1 billable unit = 10 mg).

Typical initial authorization duration: 12 months (365 days).
aGVHD: authorized up to 8 doses (4 weeks); aGVHD authorizations may NOT be renewed.
HCPCS codes for alpha‑1 products: J0256 and J0257 (1 billable unit = 10 mg).

Documentation Required

Document prior intolerance/failure to Glassia and Zemaira before other products

For consideration of any alpha-1 product other than Glassia or Zemaira, document that the patient has a contraindication, intolerance, or treatment failure to BOTH Glassia and Zemaira prior to coverage of the alternative product.

Prerequisite applies specifically to non-Glassia/Zemaira products: document contraindication, intolerance, or failure to Glassia AND to Zemaira.

Documentation Required

Document baseline criteria and disease response for renewal

For renewal, document that the member continues to meet the universal and indication‑specific criteria, that the authorization duration has not been exceeded, and that there is absence of unacceptable toxicity; for emphysema renewal include at least one disease response measure such as elevation of AAT above baseline, substantial reduction in rate of FEV1 decline, or improved CT lung density.

Confirm continued meeting of universal and indication‑specific criteria (age, contraindications, concomitant therapy, performance status).
Document absence of unacceptable toxicity (e.g., no severe hypersensitivity).
Provide at least one response measure for emphysema: increased AAT level above baseline, reduced decline in percent predicted FEV1, or improved CT lung density.

Denial Risk

Denial risk for active smoking, missing phenotype, or AAT level above threshold

Requests may be denied if the member is an active tobacco smoker, does not have an eligible AAT phenotype, or has an AAT serum concentration above the allowed threshold.

Smoking status: member must be NOT a tobacco smoker.
Required phenotypes: PiZZ, PiZ (null), or Pi (null, null) are required for emphysema coverage.
AAT serum concentration must be ≤ 57 mg/dL (nephelometry) or ≤ 11 µM/L (ELISA).

Clinical Background

Alpha-1 antitrypsin (AAT) deficiency is an inherited disorder that can result in panacinar (panlobular) emphysema. The policy covers intravenous human alpha-1-proteinase inhibitor augmentation therapyPiZZ, PiZ (null), or Pi (null, null)), clinical evidence of emphysema, and a low serum AAT concentration (≤ 57 mg/dL by nephelometry or ≤ 11 µM/L by ELISA). The covered products and HCPCS reporting include J0256 (Aralast NP, Prolastin‑C, Zemaira depending on packaging) and J0257 (Glassia), with 1 billable unit = 10 mg.

In addition to the FDA-approved emphysema indication, the policy recognizes a compendia-recommended off-label use: intravenous alpha-1-proteinase inhibitor may be considered for steroid-refractory acute graft-versus-host disease (aGVHD) following hematopoietic stem cell transplant when used in combination with systemic corticosteroids as additional therapy after failure of first‑line options. For aGVHD the policy specifies the compendia-based use and details a defined dosing schedule.

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

OpenPayer

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

Medical Necessity Criteria

Relevant Codes

What Providers Must Do

Clinical Thresholds and Dosing Limits

Clinical Background

Defined Terms

Policy Dates & Reviews

J0256	Aralast NP; Prolastin-C; Zemaira (depending on manufacturer packaging) — 1 billable unit = 10 mg
J0257	Glassia — 1 billable unit = 10 mg