Neighborhood Health Plan RI Alpha1‑PI Coverage Update

Coverage Criteria for Alpha1-Proteinase Inhibitor Augmentation Therapy

Initial Therapy

Covered when ALL of the following are met:

Initial Approval Requirements: Patient is ≥18 years old; not a tobacco smoker; receiving optimal medical therapy (e.g., comprehensive case management, pulmonary rehabilitation, vaccinations, smoking cessation, self-management); and does not have immunoglobulin‑A (IgA) deficiency with anti‑IgA antibodies.

MMP members who received this medication within the past 365 days are not subject to step therapy requirements.

Indication-specific Criteria (Emphysema due to AAT deficiency): Clinical evidence of panacinar/panlobular emphysema; AAT deficiency with phenotype PiZZ, PiZ (null), or Pi (null,null); low serum AAT concentration ≤57 mg/dL (≤11 µM/L) measured by nephelometry; and when specified, FEV1 in the range 30–65% of predicted.Serum AAT ≤57 mg/dL (≤11 µM/L); FEV1 30-65% when specified

For Commercial and MMP patients requesting Aralast or Glassia, documented failure, intolerance, or contraindication to Prolastin or Zemaira is required.

Dosing and Billing: Dose = 60 mg/kg administered by IV infusion once every 7 days (weekly); billing consistent with 10 mg = 1 billable unit and maximum 700 billable units per 7 days.60 mg/kg weekly; max 700 units/7 days

Continuation / Renewal Therapy

Renewal allowed when ALL of the following are met:

Renewal Requirements: Patient continues to meet the universal and indication‑specific criteria; demonstrates disease response to treatment as shown by elevation of AAT above baseline, substantial reduction in the rate of FEV1 decline, or improvement in CT lung density; and has absence of unacceptable toxicity (e.g., severe hypersensitivity).

Authorizations are renewed in 6‑month increments.

General medical necessity criteria

Covered when consistent with FDA-approved labeling and relevant clinical literature; Medicare NCD/LCD take precedence for Medicare members

Primary coverage rule: Use of Aralast NP, Glassia, Prolastin‑C, or Zemaira must align with FDA‑approved labeling and/or relevant clinical literature.

Neighborhood P&T Committee adopted these criteria to ensure medically accepted indications and to promote cost‑effective use.

Medicare precedence: For INTEGRITY (Medicare‑Medicaid Plan) members, these plan coverage criteria apply only in the absence of an applicable National Coverage Determination (NCD) or Local Coverage Determination (LCD).

When an NCD or LCD exists, compliance with that determination is required.

Current tobacco smoking is an explicit exclusion for coverage. Requests for alpha1‑proteinase inhibitor augmentation therapy must document that the patient is not a tobacco smoker before coverage will be approved; smoking cessation should be verified per the plan’s documentation requirements.

Patients with immunoglobulin A (IgA) deficiency who have antibodies against IgA are excluded from coverage. The universal criteria require that the patient does not have IgA deficiency with anti‑IgA antibodies documented in the medical record prior to authorization.

No additional plan‑specific exclusions are listed beyond the universal exclusions. Where applicable for Medicare members, coverage decisions are governed by existing Medicare National Coverage Determinations (NCDs), Local Coverage Determinations (LCDs), or Local Coverage Articles (LCAs), and those determinations supersede plan criteria.

Prolastin‑C is an approved Alpha1‑Proteinase Inhibitor product for augmentation therapy in adults with clinically evident emphysema due to severe hereditary Alpha1‑PI deficiency; however, the policy notes that adequately powered randomized controlled trials demonstrating clinical efficacy on pulmonary outcomes are limited. Available evidence includes a single‑arm, open‑label study showing biochemical increases in Alpha1‑PI levels with weekly 60 mg/kg dosing, while clinical outcome data remain limited.

Requests for use that are inconsistent with FDA‑approved labeling or not supported by relevant clinical literature may be considered not medically necessary. The plan will review prescriber‑submitted information and give individual consideration, but therapies lacking supportive evidence for the requested indication may be denied.

Coding, Dosing, and Billing Guidance

Covered HCPCS CodesHCPCSCovered

J0256	Alpha-1-proteinase inhibitor (human)
J0257	Alpha-1-proteinase inhibitor (human) - alternate HCPCS listed

NDC / Product AvailabilityNDC

00944-2815-xx	Aralast NP 1 g/50 mL (powder)
00944-2814-xx	Aralast NP 0.5 g/25 mL (powder)
00944-2884-xx	Aralast NP (alternate listing)
13533-0700-xx	Prolastin-C (powder) NDC series
13533-0701-xx	Prolastin-C (powder) NDC series
13533-0702-xx	Prolastin-C (powder) NDC series
13533-0703-xx	Prolastin-C (powder) NDC series
13533-0705-xx	Prolastin-C Liquid (solution)
00053-7201-xx	Zemaira 1 g/20 mL (powder)
00053-7202-xx	Zemaira 4 g/76 mL (powder)

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Covered ICD-10 CodesICD-10Covered

E88.01

Alpha-1-antitrypsin deficiency

Product NDCs and Quantity Limits (per week)NDC

Aralast NP 1 g/50 mL	NDC 00944-2815-xx — 7 vials per week
Aralast NP 0.5 g/25 mL	NDC 00944-2814-xx — 1 vial per week
Glassia 1 g/50 mL	(NDC not listed) — 3 vials per week
Glassia 4 g/150 mL	(NDC not listed) — 2 vials per week
Glassia 5 g/250 mL	(NDC not listed) — 2 vials per week
Prolastin-C 1 g/20 mL	NDC 13533-0700-xx / 13533-0701-xx / 13533-0702-xx / 13533-0703-xx — 7 vials per week
Prolastin-C Liquid 1 g/20 mL	NDC 13533-0705-xx — 7 vials per week
Zemaira 1 g/20 mL	NDC 00053-7201-xx — 3 vials per week
Zemaira 4 g/76 mL	NDC 00053-7202-xx — 1 vial per week
Zemaira 5 g/95 mL	NDC 00053-7203-xx — 1 vial per week

Medicare Part B Covered Diagnosis CodesICD-10

No codes listed

Medicare Part B Administrative Contractor (MAC) Jurisdictionsmixed

Noridian Healthcare Solutions, LLC	Jurisdictions: CA, HI, NV, AS, GU, CNMI; AK, WA, OR, ID, ND, SD, MT, WY, UT, AZ
Wisconsin Physicians Service Insurance Corp (WPS)	Jurisdictions: KS, NE, IA, MO; MI, IN
National Government Services, Inc. (NGS)	Jurisdictions: MN, WI, IL; NY, CT, MA, RI, VT, ME, NH
Novitas Solutions, Inc.	Jurisdictions: LA, AR, MS, TX, OK, CO, NM; DE, MD, PA, NJ, DC
First Coast Service Options, Inc.	Jurisdictions: FL, PR, VI
Palmetto GBA, LLC	Jurisdictions: TN, GA, AL; NC, SC, WV, VA
CGS Administrators, LLC	Jurisdictions: KY, OH

Dose and billing guidance

Dose and billing thresholdDose = 60 mg/kg weekly; Billing threshold = 700 billable units per 7 days (1 unit = 10 mg)

Clinical thresholds

Serum AAT threshold≤57 mg/dL or ≤11 µM/L by nephelometry

FEV1 inclusion range30-65% predicted

Provider Actions, Prior Authorization, and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization is required for coverage. Initial authorization requires documentation that the member meets age, universal, and indication-specific criteria. MMP members who have previously received the medication within the past 365 days are not subject to step therapy requirements. For Medicare (INTEGRITY) members, plan criteria apply only in the absence of an applicable CMS National Coverage Determination (NCD) or Local Coverage Determination (LCD); Medicare NCDs/LCDs and related policies take precedence where applicable.

Prior authorization required for initial and renewal requests.
MMP prior-use exception: prior receipt within 365 days exempts member from step therapy.
Medicare precedence: comply with NCD/LCD where applicable; plan criteria apply only when no NCD/LCD exists.

Documentation Required

Diagnostic Phenotype and Level

Coverage for emphysema due to alpha-1-antitrypsin (AAT) deficiency requires documentation of the diagnostic phenotype and serum level consistent with FDA-approved labeling: phenotypes PiZZ, PiZ (null), or Pi (null, null) and a low serum AAT concentration ≤ 57 mg/dL (≤ 11 µM/L) measured by nephelometry. Also document clinical evidence of panacinar/panlobular emphysema and FEV1 in the specified range when applicable.

Background

Alpha1‑proteinase inhibitor (Alpha1‑PI) augmentation therapy is indicated to augment and maintain serum Alpha1‑PI levels in adults with clinically evident emphysema due to severe hereditary Alpha1‑PI deficiency. Clinical trials for the approved products demonstrated that weekly intravenous infusions at 60 mg/kg once every 7 days increase serum and epithelial lining fluid Alpha1‑PI concentrations. The Neighborhood P&T Committee reviewed Aralast NP, Glassia, Prolastin‑C, and Zemaira and adopted coverage criteria aligning use with FDA labeling and relevant literature to ensure medically accepted indications and consideration of cost‑effectiveness.

Definitions and Key Terms

Alpha1-PI (Alpha1-Proteinase Inhibitor)

DefinitionAlpha1-Proteinase Inhibitor (Alpha1-PI): a human plasma-derived protein used for chronic augmentation and maintenance therapy in adults with clinically evident emphysema due to severe hereditary or congenital deficiency of Alpha1-PI.

Therapeutic goalAugment and maintain serum and epithelial lining fluid A1-PI levels to above target concentrations demonstrated in clinical trials.

Common products reviewedAralast NP, Glassia, Prolastin-C, Zemaira

Covered genotypes and low serum threshold

Covered severe AAT phenotypesPiZZ; PiZ (null); Pi (null, null)

Low serum AAT definitionSerum AAT concentration ≤57 mg/dL (≤11 µM/L) measured by nephelometry

OpenPayer

Alpha1-Proteinase Inhibitor (AAT) augmentation therapy (Aralast NP, Glassia, Prolastin-C, Zemaira)

Coverage Criteria for Alpha1-Proteinase Inhibitor Augmentation Therapy

Coding, Dosing, and Billing Guidance

Provider Actions, Prior Authorization, and Documentation

Background

Definitions and Key Terms