CurrentNeighborhood Health Plan of Rhode IslandPolicy 1877-A

Alpha1-Proteinase Inhibitor Therapy (AAT augmentation)

Policy governing prior authorization and coverage criteria for chronic augmentation/maintenance therapy with alpha1-proteinase inhibitors for members with emphysema due to severe alpha1-antitrypsin deficiency. Affects providers prescribing Aralast NP, Glassia, Prolastin-C, Zemaira for Neighborhood Health Plan of Rhode Island members.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyAlpha1-Proteinase Inhibitor Therapy (AAT augmentation)

Policy CodePolicy 1877-A

Change TypeNo material change

Effective DateN/A

Next Review DateN/A

Key ActionSubmit pretreatment post-bronchodilation FEV1, pretreatment serum AAT level, and AAT protein phenotype or genotype for prior authorization.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12 monthsauthorization period

<11 μmol/Lserum AAT threshold

FEV1 requiredFEV1 documented

PiZZ/null homozygoteseligible genotypes

PiMZ/PiMSexcluded phenotypes

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Coverage Criteria for Alpha1-Proteinase Inhibitor (AAT) Therapy

Initial Therapy

Covered when ALL of the following are met:

Initial approval

Documentation: Submission includes pretreatment post-bronchodilation FEV1, pretreatment serum AAT level, and AAT protein phenotype or genotype.
See required documentation for prior authorization; pretreatment post-bronchodilation FEV1 and AAT measurements must be provided.
Serum AAT: Pretreatment serum AAT level is less than 11 micromol/L (80 mg/dL by radial immunodiffusion or 50 mg/dL by nephelometry).< 11 micromol/L (80 mg/dL RID; 50 mg/dL nephelometry)
Genotype/Phenotype: Documented PiZZ, PiZ (null), or Pi (null,null) (homozygous) AAT deficiency or other phenotype/genotype associated with serum AAT < 11 micromol/L.
Members with PiMZ or PiMS phenotypes are not eligible for approval.

Continuation Therapy

Covered for continuation when ALL of the following are met:

Continuation: Member has been previously authorized for AAT augmentation therapy and is experiencing a beneficial clinical response from therapy.

Authorization of up to 12 months may be granted for continued treatment when criteria are met.

Members with PiMZ or PiMS alpha1-antitrypsin (AAT) deficiency phenotypes are not eligible for approval for augmentation therapy with alpha1-proteinase inhibitors.

Only the listed FDA-approved and compendial indications for chronic augmentation/maintenance therapy in adults with clinically evident emphysema due to severe AAT deficiency are considered a covered benefit. All other indications are considered experimental/investigational and not medically necessary and therefore are not covered.

Covered Products and Lab Thresholds

inv-06: Serum AAT concentration — thresholds and measurement equivalents

Serum AAT threshold (primary)< 11 micromol/L

Equivalent by radial immunodiffusion (RID)80 mg/dL

Equivalent by nephelometry50 mg/dL

Measurement contextPretreatment serum AAT level (used for eligibility)

Prior Authorization, Documentation, and Process

Prior Authorization

Prior Authorization Required

Prior authorization is required for alpha1-proteinase inhibitor therapy. When criteria are met, authorization may be granted for up to 12 months.

Authorization duration: 12 months when clinical criteria are satisfied
Prior authorization required before initiation of therapy

Step Therapy

No step therapy requirements are specified for alpha1-proteinase inhibitors in this policy.

Documentation Required

Background

Alpha1-antitrypsin deficiency (AATD) is a hereditary condition in which low serum levels of AAT can lead to clinically evident emphysema. Chronic augmentation therapy with human plasma–derived alpha1-proteinase inhibitors is used as maintenance treatment in selected adults with emphysema due to severe congenital or hereditary AAT deficiency to slow progression of lung disease.

Coverage for augmentation therapy is limited to members with documented severe deficiency — typically a pretreatment serum AAT concentration of < 11 micromol/L (80 mg/dL by radial immunodiffusion or 50 mg/dL by nephelometry) — and an eligible deficient genotype/phenotype such as PiZZ, PiZ (null), or Pi (null,null) (homozygous). Treatment decisions also require documentation of pretreatment post-bronchodilation FEV1. Phenotypes such as PiMZ and PiMS are excluded from eligibility.

Definitions

inv-12: Severe AAT deficiency — definition and clinical significance

Definition (numeric)Serum AAT concentration less than 11 micromol/L (80 mg/dL by radial immunodiffusion or 50 mg/dL by nephelometry)

Associated genotypes/phenotypesDocumented PiZZ, PiZ (null), or Pi (null,null) (homozygous) AAT deficiency or other phenotype/genotype associated with AAT < 11 micromol/L

Clinical significanceIndicates severe congenital/hereditary AAT deficiency associated with clinically evident emphysema and eligibility for augmentation therapy when other criteria met

ExclusionsPhenotypes PiMZ and PiMS are not eligible for approval despite AAT testing