CurrentViva HealthPolicy IC-0052

Alpha-1-Proteinase Inhibitors (Intravenous)

Prior authorization policy for intravenous alpha-1-proteinase inhibitor products (Aralast NP, Glassia, Prolastin-C, Zemaira) for indications including emphysema due to AAT deficiency and acute graft‑versus‑host disease, governing Viva Health members and providers submitting PA requests.

Policy Summary

PayerViva Health

PolicyAlpha-1-Proteinase Inhibitors (Intravenous)

Policy CodePolicy IC-0052

Change TypeNo material changes

Effective Date

Next Review Date

Key ActionObtain prior authorization; initial approvals generally valid 12 months (aGVHD limited to up to 8 doses/4 weeks).

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12 monthsinitial prior authorization validity

8 dosesmax PA validity for aGVHD

60 mg/kgstandard dose

30-65%FEV1 requirement

≤57 mg/dLAAT threshold

Coverage Criteria

Trek Health ingests and normalizes Transparency in Coverage data and payer policy updates to give provider organizations a clear view of how commercial reimbursement behaves across markets, payers, and services. Our platform transforms raw payer disclosures into structured intelligence that supports contract evaluation, payer negotiations, and service line strategy. By combining market benchmarks with ongoing policy visibility, Trek helps teams identify variability, risk, and opportunity in commercial reimbursement. The result is faster insight, stronger negotiating positions, and more informed financial decisions.

Initial Approval — Emphysema due to AAT deficiency

Covered when ALL of the following are met

Universal and indication-specific criteria: Member is ≥18 years of age; member does NOT have any FDA-labeled contraindications to the requested agent; FEV1 30-65% of predicted; presence of alpha-1-antitrypsin (AAT) deficiency with PiZZ, PiZ (null), or Pi (null,null) phenotype; clinical evidence of panacinar/panlobular or centrilobular emphysema; serum AAT ≤ 57 mg/dL (measured by nephelometry) or ≤ 11 µM/L (measured by ELISA); member is not a tobacco smoker; member is receiving optimal medical therapy (e.g., bronchodilators, inhaled corticosteroids, pulmonary rehabilitation, vaccinations, smoking cessation, supplemental oxygen, etc.); documentation of contraindication, intolerance, or failure to Glassia AND Zemaira prior to consideration of another alpha-1 proteinase inhibitor product.

Dose for AAT deficiency: 60 mg/kg IV once weekly.

Initial Approval — Acute Graft Versus Host Disease (aGVHD)

Covered when ALL of the following are met

aGVHD criteria: Member has received a hematopoietic stem cell transplant; indication is steroid-refractory acute graft-versus-host disease (aGVHD); used in combination with systemic corticosteroids as additional therapy following no response to first-line therapy options; duration limited to a maximum of 8 doses over 28 days.

Dose: 60 mg/kg IV on days 1, 4, 8, 12, 16, 20, 24, and 28. Prior authorization valid for up to 8 doses (4 weeks) and may not be renewed.

Coverage is not provided for patients who do not meet the universal AAT deficiency clinical criteria. Specifically, members who are tobacco smokers; those without the required phenotypes (PiZZ, PiZ (null), or Pi (null,null)); or those with a serum AAT level > 57 mg/dL (>11 µM/L) do not meet the policy criteria. Additionally, for AAT deficiency, use of an alternative alpha-1 proteinase inhibitor will only be considered after documentation of contraindication, intolerance, or failure to both Glassia and Zemaira prior to the request.

Prior authorization may be renewed only if the member continues to meet the universal and indication-specific criteria in Section III (including concomitant therapy requirements and performance status), there is no unacceptable drug toxicity, and the duration of authorization has not been exceeded. Examples of unacceptable toxicity include severe hypersensitivity reactions. For acute graft-versus-host disease (aGVHD), initial authorization is limited to a maximum of 8 doses (over 28 days) and may not be renewed.

Coding and Clinical Thresholds

HCPCS CodesHCPCS

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

Covered ICD-10 Diagnosis CodesICD-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

inv-07: Serum AAT level — threshold value

Serum AAT threshold≤ 57 mg/dL (nephelometry) OR ≤ 11 µM/L (ELISA)

Measurement methodsNephelometry (mg/dL) or ELISA (µM/L)

Coverage requirementMember must have low serum AAT at or below above thresholds to meet AAT deficiency criteria

inv-08: FEV1 percent predicted — clinical range

FEV1 percent predicted range30-65% of predicted

Role in eligibilityRequired for AAT deficiency coverage as part of universal criteria

Clinical contextFEV1 within this range indicates appropriate lung function severity for augmentation therapy

Provider Actions and Authorization

Prior Authorization

Prior Authorization Required

Prior authorization is required. Initial approvals are provided for 12 months (365 days) unless otherwise specified. For acute graft-versus-host disease (aGVHD), prior authorization validity is provided for a maximum of 8 doses (4 weeks) and may NOT be renewed.

Initial PA validity: 12 months (365 days)
aGVHD: up to 8 doses (4 weeks); not renewable

Step Therapy

Step Therapy: Glassia and Zemaira Required

For alpha-1 antitrypsin (AAT) deficiency, the member must have a documented contraindication, intolerance, or failure to BOTH Glassia and Zemaira before other A1PI products will be considered.

Required prior use or documented intolerance/contraindication/failure to Glassia and Zemaira
Applies to initiation of other alpha-1 proteinase inhibitor products

Documentation Required

Submit documentation that supports the prior use and intolerance/contraindication/failure to Glassia and Zemaira, and evidence addressing the universal and disease-specific criteria. Lack of required documentation may result in denial.

Clinical documentation must include: prior trial records for Glassia and Zemaira, AAT phenotype, serum AAT level (method and value), FEV1 percent predicted, smoking status, and evidence of emphysema type and prior/concurrent therapies.
Incomplete documentation is a denial risk

Denial Risk

Clinical Criteria Not Met — Denial Risk

The request may be denied if clinical criteria are not met. Examples of denial-risk conditions include: tobacco use, serum AAT level > 57 mg/dL, phenotypes not meeting PiZZ/PiZ (null)/Pi(null,null) criteria, FEV1 outside the 30–65% predicted range, or presence of FDA-labeled contraindications to the requested agent.

Tobacco smoker (current) — not eligible
Serum AAT > 57 mg/dL (nephelometry) or > 11 µM/L (ELISA) — not eligible
Phenotype not PiZZ, PiZ (null), or Pi (null,null) — not eligible
FEV1 < 30% or > 65% predicted — not eligible
FDA-labeled contraindications to the requested agent — not eligible

Definitions and Indication Notes

inv-14: AAT deficiency criteria — phenotype, serum threshold, FEV1 range, clinical evidence

Phenotype requiredPiZZ, PiZ (null), or Pi (null,null)

Serum AAT level≤ 57 mg/dL (nephelometry) or ≤ 11 µM/L (ELISA)

FEV1 range30-65% of predicted

Clinical evidenceClinical evidence of panacinar/panlobular or centrilobular emphysema; member must be a non-smoker and receiving optimal medical therapy

inv-15: aGVHD use — indication summary for steroid-refractory acute graft-versus-host disease

Indication summaryUse for steroid-refractory acute graft-versus-host disease (aGVHD) in patients who have received a hematopoietic stem cell transplant

Use with corticosteroidsAdministered in combination with systemic corticosteroids as additional therapy after no response to first-line options

SettingCompendia-recommended indication for post-transplant steroid-refractory aGVHD

Background

Alpha-1 antitrypsin (AAT) deficiency can lead to panacinar or panlobular emphysema. Intravenous augmentation with human alpha-1 proteinase inhibitor products is indicated for selected adult patients who demonstrate documented AAT deficiency (phenotypes such as PiZZ, PiZ (null), or Pi (null,null)), low serum AAT concentration (≤ 57 mg/dL or ≤ 11 µM/L), and appropriate lung function (FEV1 in the range of 30–65% predicted) with clinical evidence of emphysema despite optimal medical therapy. For non-emphysema use, these agents are also referenced for steroid-refractory acute graft-versus-host disease in hematopoietic stem cell transplant recipients when used with systemic corticosteroids.