CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Alpha1‑Proteinase Inhibitor (intravenous) therapy (Aralast NP, Glassia, Prolastin‑C, Zemaira)

Covers clinical criteria, dosing, authorization, and billing for IV alpha‑1‑proteinase inhibitor augmentation therapy for members of Neighborhood Health Plan of Rhode Island across Medicaid, Commercial, and Medicare‑Medicaid plans.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyAlpha1‑Proteinase Inhibitor (intravenous) therapy (Aralast NP, Glassia, Prolastin‑C, Zemaira)

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateJan 1, 2020

Next Review DateJan 10, 2024

Key ActionObtain prior authorization; patient must be age ≥18 and meet clinical criteria (non‑smoker, appropriate phenotype/low AAT level, FEV1 range) for coverage.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6 monthsstandard authorization period

60 mg/kg weeklyrecommended dose

700max HCPCS units/7d

≥18minimum age

E88.01covered diagnosis

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Coverage Criteria

Initial Approval Criteria

Initial Approval — Covered when ALL of the following are met

Initial approval criteria: Patient is at least 18 years of age; patient is not a tobacco smoker; patient is receiving optimal medical therapy (e.g., comprehensive case management, pulmonary rehabilitation, vaccinations, smoking cessation, self-management skills); patient does not have immunoglobulin‑A (IgA) deficiency with antibodies against IgA; indication‑specific criteria below must be met.

Universal criteria

Emphysema due to AAT deficiency (indication‑specific): Patient has an FEV1 in the range of 30–65% of predicted; patient has AAT deficiency with PiZZ, PiZ (null), or Pi (null, null) phenotypes; patient has clinical evidence of panacinar/panlobular emphysema; patient has low serum AAT concentration ≤ 57 mg/dL (≤ 11 µM/L) measured by nephelometry; for Commercial and MMP patients requesting Aralast or Glassia, documented failure, intolerance, or contraindication to Prolastin or Zemaira is required.FEV1 30–65% predicted; AAT ≤ 57 mg/dL (≤ 11 µM/L)

Applies to FDA‑approved indication; Prolastin‑C specific note in source

Renewal Criteria

Renewal — Covered when ALL of the following are met

Renewal criteria: Patient continues to meet universal and indication‑specific criteria; demonstrates disease response defined by elevation of AAT levels above baseline, substantial reduction in rate of deterioration of lung function as measured by percent predicted FEV1, or improvement in CT scan lung density; and there is an absence of unacceptable toxicity from the drug.

Authorizations can be renewed for 6 months

General coverage criteria

Covered when ALL of the following are met (policy-level summary):

Plan-level coverage conditions

Diagnosis: Member has diagnosis code E88.01 (Alpha‑1‑antitrypsin deficiency)
See Appendix 1

Cost-effectiveness / step therapy: More cost‑effective options should be used first, if applicable and appropriate.

Implies step therapy per plan criteria; specifics addressed elsewhere in policy.

Requests for alpha‑1‑proteinase inhibitor augmentation therapy are excluded when the patient is an active tobacco smoker. The policy requires that the patient must not be a tobacco smoker as part of the universal criteria for coverage.

Per the document appendix referencing Medicare guidance, there are no additional Medicare Part B covered diagnosis codes listed for this therapy under existing NCD/LCD/LCA policies (listed as N/A); providers should follow applicable CMS coverage determinations where present.

Coverage is limited to patients with severe hereditary AAT deficiency phenotypes (for example, PiZZ, PiZ (null), or Pi (null,null)) and who have clinical evidence of panacinar/panlobular emphysema. Requests for use in patients who do not meet these phenotype criteria, lack clinical evidence of panacinar/panlobular disease, or do not have a low serum AAT concentration (≤ 57 mg/dL or ≤ 11 µM/L by nephelometry) do not meet the specified indication.

Therapy requests that do not conform to the plan’s clinical coverage criteria based on FDA labeling or relevant clinical literature may be considered not medically necessary. Neighborhood will give individual consideration to requests when prescriber‑submitted clinical information supports an FDA‑approved indication or relevant literature justification.

Coding and Billing

Covered HCPCS CodesHCPCSCovered

J0256	Alpha-1 Proteinase Inhibitor (Human), non lyophilized powder, 10 mg
J0257	Alpha-1 Proteinase Inhibitor (Human), lyophilized, 10 mg

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

E88.01

Alpha-1-antitrypsin deficiency

Product NDC / Packaging (NDC)NDC

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 packaging, powder
13533-0700-xx	Prolastin-C, powder, packaging
13533-0701-xx	Prolastin-C, powder, packaging
13533-0702-xx	Prolastin-C, powder, packaging
13533-0703-xx	Prolastin-C, powder, packaging
13533-0705-xx	Prolastin-C, liquid (solution), packaging
00053-7201-xx	Zemaira, powder, packaging
00053-7202-xx	Zemaira, powder, packaging

1–10 of 11

1/2

Alternate Covered Diagnosis CodesICD-10Covered

E88.01

Alpha-1-antitrypsin deficiency

Medicare Part B Covered Diagnosis CodesICD-10

No codes listed

HCPCS units / dosing frequency

HCPCS units / dosing frequency700 billable units every 7 days; Dose = 60 mg/kg IV once every 7 days

Provider Actions and Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for Aralast NP, Glassia, Prolastin‑C, and Zemaira per plan policy. Members of the Medicare‑Medicaid Plan (MMP) who have received the medication within the past 365 days are exempt from step‑therapy requirements.

Prior authorization required
MMP members with prior receipt within 365 days exempt from step therapy

Denial Risk

Denial Risk for Unsupported Requests

Neighborhood will give individual consideration to requests based on clinical information submitted by the prescriber. Requests that do not meet plan criteria developed from FDA labeling and relevant literature may be denied.

Denial risk for unsupported requests — requests lacking documentation to meet FDA‑based criteria or relevant literature may be denied
Individual consideration depends on prescriber‑submitted clinical information

Background

Alpha1‑proteinase inhibitor (Alpha1‑PI) augmentation therapy is indicated for adults with clinically evident emphysema due to severe hereditary alpha‑1‑antitrypsin deficiency. The plan’s P&T committee reviewed products including Aralast NP, Glassia, Prolastin‑C, and Zemaira and adopted criteria to align use with FDA‑approved labeling and relevant literature. Universal coverage requirements include that the patient is ≥ 18 years old, is not a tobacco smoker, is receiving optimal medical therapy, and does not have IgA deficiency with anti‑IgA antibodies. For the emphysema indication, covered patients typically have an FEV1 of 30–65% predicted, a qualifying severe phenotype (e.g., PiZZ), clinical evidence of panacinar/panlobular emphysema, and a low serum AAT level (≤ 57 mg/dL or ≤ 11 µM/L). Typical dosing for augmentation therapy is 60 mg/kg IV once every 7 days.

Definitions

Protective serum level

Protective trough concentrationTarget trough serum Alpha1‑PI concentration >= 11 µM (≈ 80 mg/dL)

Evidence from trialsClinical trials for Aralast NP, Glassia, Prolastin‑C, and Zemaira demonstrated maintenance of trough levels at or above ~11 µM with 60 mg/kg weekly dosing

Clinical relevanceTrough >=11 µM associated with increased Alpha1‑PI in epithelial lining fluid in multiple studies

Covered diagnosis (ICD-10)

ICD‑10 codeE88.01

DescriptionAlpha-1-antitrypsin deficiency

Usage