RetiredUnitedHealthcarePolicy CSLA2025D0067J

Alpha1-Proteinase Inhibitors (for Louisiana Only) – Community Plan Medical Benefit Drug Policy

UnitedHealthcare Community Plan medical benefit drug policy, Louisiana-only, governing coverage criteria for alpha1-proteinase inhibitor products (Aralast NP, Glassia, Prolastin-C, Zemaira) for chronic augmentation/maintenance therapy in patients with emphysema due to congenital A1-PI (AAT) deficiency. Policy was retired effective April 1, 2026.

Policy Summary

PayerUnitedHealthcare

PolicyAlpha1-Proteinase Inhibitors (for Louisiana Only) – Community Plan Medical Benefit Drug Policy

Policy CodePolicy CSLA2025D0067J

Change TypeRetired

Effective DateNov 1, 2025

Next Review Date

Key ActionInitial authorization limited to no more than 12 months and requires documentation of phenotype/genotype confirmation, serum AAT level < 11 µmol/L, confirmation of emphysema by PFT, nonsmoker status, continued optimal conventional treatment, and FDA-consistent dosing.

SourceLink

POLICY UPDATE CHANGES

Policy retired; Louisiana plan membership disenrolled on Apr. 1, 2026.

Updated supporting information and references on 11/01/2025 and archived previous policy version CSLA2024D0067I.

4FDA-labeled products referenced

1Covered indication (AAT deficiency emphysema)

2Unproven indications listed (CF and other)

Coverage Summary & Scope

UnitedHealthcare Community Plan medical benefit drug policy for Louisiana only governing alpha1-proteinase inhibitors (Aralast NP, Glassia, Prolastin-C, Zemaira) for chronic augmentation/maintenance therapy. The policy covers augmentation/maintenance therapy for emphysema due to congenital alpha1-proteinase inhibitor (A1-PI / AAT) deficiency. This policy was retired effective April 1, 2026 and Louisiana plan membership was disenrolled on that date.

Key threshold and quick facts

Serum AAT concentration threshold< 11 µmol/L (< 80 mg/dl by radial immunodiffusion or < 57 mg/dl by nephelometry)

FDA-labeled products referenced4

Covered indication count1 (emphysema due to congenital A1-PI/AAT deficiency)

Unproven indications listed2 (conditions other than emphysema; cystic fibrosis)

Initial Therapy (Medically Necessary) Criteria

Initial therapy (medically necessary)

Covered when ALL of the following are met:

Diagnosis of congenital alpha1-antitrypsin (AAT) deficiency confirmed by one of the following protein phenotypes: Pi*ZZ, Pi*Z(null) or Pi*(null)(null) (homozygous)
Other disease-causing alleles: Other rare AAT deficiency disease-causing alleles associated with serum AAT level < 11 µmol/L (e.g., Pi (Malton, Malton))< 11 µmol/L
Serum AAT concentration: Circulating serum concentration of AAT < 11 µmol/L (which corresponds to < 80 mg/dl if measured by radial immunodiffusion or < 57 mg/dl if measured by nephelometry)< 11 µmol/L (< 80 mg/dl by radial immunodiffusion or < 57 mg/dl by nephelometry)
Conventional treatment: Continued optimal conventional treatment for emphysema (e.g., bronchodilators, supplemental oxygen if necessary)
Smoking status: Current nonsmoker
Emphysema confirmation: Diagnosis of emphysema confirmed with pulmonary function testing
Dosing is in accordance with the United States Food and Drug Administration approved labeling

Prior Authorization

Initial authorization requirement

Initial authorization is limited to no more than 12 months. Required documentation includes confirmation of qualifying phenotype/genotype (e.g., Pi*ZZ or other disease‑causing allele associated with AAT deficiency), circulating serum AAT concentration < 11 µmol/L (specify assay method and corresponding thresholds), demonstration of emphysema by pulmonary function testing, evidence of continued optimal conventional treatment, current nonsmoker status, and dosing consistent with FDA‑approved labeling. Affected HCPCS codes: J0256, J0257.

J0256
J0257

Continuation / Reauthorization Criteria

Continuation of therapy (medically necessary)

Covered when ALL of the following are met:

Current therapy: Patient is currently receiving therapy for emphysema due to congenital alpha1-antitrypsin (AAT) deficiency
Clinical response: Documentation of a positive clinical response (e.g., decreased frequency of exacerbations, improvement in symptom burden, slowed rate of FEV1)
Smoking status: Patient remains a nonsmoker
Dosing is in accordance with the United States Food and Drug Administration approved labeling

Prior Authorization

Reauthorization requirement

Reauthorization is limited to no more than 12 months. Required documentation includes that the patient is currently receiving therapy for emphysema due to congenital AAT deficiency, evidence of a positive clinical response (for example, decreased exacerbation frequency, improved symptoms, or slowed FEV1 decline), continued nonsmoker status, and dosing consistent with FDA‑approved labeling. Affected HCPCS codes: J0256, J0257.

J0256
J0257

Not Covered / Unproven Indications

Not covered / Unproven indications

Alpha1-proteinase inhibitors are unproven for:

Other conditions: Conditions other than emphysema associated with alpha1-antitrypsin deficiency
Cystic fibrosis

Denial Risk

Not indicated for other conditions

Claims for indications other than emphysema due to congenital A1‑PI (AAT) deficiency — including cystic fibrosis and other non‑emphysema conditions — are considered unproven and may be denied.

Provider Actions & Documentation Requirements

Documentation Required

Diagnostic confirmation

Documentation must include genotype or protein phenotype (for example, PiZZ or other disease‑causing allele) and objective serum AAT level with the method of assay specified (radial immunodiffusion or nephelometry and corresponding thresholds), and pulmonary function testing confirming emphysema.

Initial and reauthorization approvals are limited to no more than 12 months. Requests require documentation of continued optimal conventional treatment and that the patient is a current nonsmoker. Dosing must be consistent with the FDA-approved labeling. Relevant HCPCS codes for claims and prior authorization are J0256 and J0257.

Evidence Summary

47.6Adjusted mean FEV1 benefit with augmentation (mL/yr) — Tonelli et al.

49.2→34.2Mean FEV1 decline reduced during treatment (mL/yr) — Multicenter retrospective cohort

RR 0.64Mortality reduction associated with augmentation (risk ratio, NHLBI registry)

Clinical evidence includes observational cohort studies and registry analyses suggesting augmentation therapy raises AAT levels and is associated with a slowed decline in FEV1 and possible mortality benefit in some analyses. However, randomized controlled trial evidence demonstrating effects on emphysema progression or long-term clinical outcomes is limited, and the FDA notes that effects on pulmonary exacerbations and emphysema progression have not been demonstrated in randomized, controlled trials.

Background & Definitions

Alpha1-PI deficiency is an autosomal, co-dominant hereditary disorder with reduced A1-PI levels in blood and lung, leading to increased neutrophil elastase activity and destruction of lung parenchyma. Augmentation therapy increases serum and lung AAT levels, restoring anti-neutrophil elastase capacity and thereby theoretically protecting the lower respiratory tract from protease-mediated damage.

Term	Definition
AAT	Alpha1-antitrypsin, also called alpha1-proteinase inhibitor (A1-PI)
PiZZ	Homozygous severe AAT deficiency phenotype (common genotype qualifying for therapy)