Currentculinary health fundPolicy N/A

Alpha1-Proteinase Inhibitor Products (AAT augmentation therapy)

Prior authorization and medical necessity criteria for alpha1-proteinase inhibitor products (Aralast NP, Glassia, Prolastin-C, Prolastin-C Liquid, Zemaira) for adults with AAT deficiency-related emphysema or panniculitis under Culinary Health Fund.

Policy Summary

Payerculinary health fund

PolicyAlpha1-Proteinase Inhibitor Products (AAT augmentation therapy)

Policy CodePolicy N/A

Change TypeMaterial criteria additions (initial & continuation requirements)

Effective Date

Next Review DateDec 3, 2025

Key ActionObtain and document baseline (pretreatment) serum AAT < 11 mcM and genotype/phenotype showing disease-causing alleles when requesting authorization.

SourceLink

POLICY UPDATE CHANGES

For initial therapy, requirement added that the patient had genotyping or phenotyping demonstrating ZZ, (null)(null), Z(null), SZ or other rare disease-causing alleles associated with serum AAT level < 11 mcM; prescriber must attest to clinical evidence of emphysema or COPD; medication prescribed by or in consultation with a pulmonologist.

For AAT deficiency-associated panniculitis initial therapy, added requirements for skin-biopsy confirmation, baseline AAT serum concentration < 11 mcM, genotyping/phenotyping for disease-causing alleles, and prescription by or in consultation with a dermatologist or pulmonologist.

For patients currently receiving therapy, requirement added for documentation of baseline (pretreatment) AAT serum concentration < 11 mcM.

5commercially available AAT products listed

60 mg/kg weeklyapproved dosing for adults (IV)

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Coverage and Medical Necessity Criteria

Alpha1-Antitrypsin Deficiency with Emphysema (or COPD)

Covered when ONE of the following (A or B) is met for adults:

A) Initial Therapy

i. Patient is ≥ 18 years of age;
ii. Baseline (pretreatment) alpha1-antitrypsin serum concentration < 11 mcM (11 micromol/L) [< 80 mg/dL if radial immunodiffusion or < 57 mg/dL if nephelometry];
iii. Genotyping or phenotyping demonstrates one of: ZZ, (null)(null), Z(null), SZ, or other rare disease-causing alleles associated with AAT < 11 mcM;
iv. According to the prescriber, the patient has clinical evidence of emphysema or chronic obstructive pulmonary disease;
v. According to the prescriber, the patient is a current non-smoker;
vi. Medication is prescribed by or in consultation with a pulmonologist.

B) Patient Currently Receiving an Alpha1-Proteinase Inhibitor Product (Continuation Therapy)

i. Patient is ≥ 18 years of age;
ii. Patient had a baseline (pretreatment) alpha1-antitrypsin serum concentration of < 11 mcM (11 micromol/L) [< 80 mg/dL RID or < 57 mg/dL nephelometry];
iii. According to the prescriber, the patient is a current non-smoker.

Alpha1-Antitrypsin Deficiency-Associated Panniculitis

Covered when ONE of the following (A or B) is met for adults:

A) Initial Therapy

i. Patient is ≥ 18 years of age;
ii. Diagnosis of panniculitis confirmed by skin biopsy;
iii. Baseline (pretreatment) alpha1-antitrypsin serum concentration < 11 mcM (11 micromol/L) [< 80 mg/dL RID or < 57 mg/dL nephelometry];
iv. Genotyping or phenotyping demonstrates one of: ZZ, (null)(null), Z(null), SZ, or other rare disease-causing alleles associated with AAT < 11 mcM;
v. Medication is prescribed by or in consultation with a dermatologist or pulmonologist.

Coverage of alpha1-proteinase inhibitor products is not recommended for individuals with alpha1-antitrypsin (AAT) deficiency without lung disease, even when deficiency-induced hepatic disease is present. The ATS/ERS guidance notes the pathophysiology of liver disease differs from lung disease and does not support use of augmentation therapy for hepatic manifestations. Coverage also is not recommended for situations not listed in the Recommended Authorization Criteria; such requests are expected to be denied.

Use of alpha1-proteinase inhibitor products for COPD unrelated to alpha1-antitrypsin deficiency is considered not medically necessary and is not recommended for coverage. Guidelines and evidence do not support augmentation therapy for COPD in the absence of documented AAT deficiency.

Products, Dosing, and Key Clinical Thresholds

Covered NDC CodesNDCCovered

00074-0430-01	Aralast NP 500 mg vial (example NDC - consult product labeling for exact pack sizes)
00074-0431-01	Aralast NP 1000 mg vial (example NDC)
54868-0250-01	Glassia 100 mg/mL 10 mL vial (example NDC)
54868-0300-01	Glassia 200 mg/mL 10 mL vial (example NDC)
58118-0030-01	Prolastin-C 500 mg vial (example NDC)
58118-0031-01	Prolastin-C 1000 mg vial (example NDC)
64679-0120-01	Prolastin-C Liquid 1200 mg/20 mL (example NDC)
52959-0100-01	Zemaira 1000 mg vial (example NDC)
52959-0101-01	Zemaira 500 mg vial (example NDC)

Serum alpha1-antitrypsin concentration — protective/eligibility threshold and assay equivalencies

Serum alpha1-antitrypsin concentration — protective/eligibility threshold and assay equivalenciesThreshold for treatment eligibility: < 11 mcM (11 micromol/L). Equivalent assay values: < 80 mg/dL if measured by radial immunodiffusion; < 57 mg/dL if measured by nephelometry.

Age requirement for approvals

Age requirement for approvalsPatient must be ≥ 18 years of age for both emphysema/COPD and panniculitis indications.

Dosing — approved adult IV dosing

Dosing — approved adult IV dosingApproved dose: 60 mg/kg administered intravenously once weekly for both emphysema/COPD and AAT deficiency–associated panniculitis (product labeling recommends 60 mg/kg once weekly).

Prior Authorization, Documentation, and Denial Risks

Prior Authorization

Prior Authorization Required

Prior Authorization is required for alpha1-proteinase inhibitor products. Approvals are typically issued for 1 year for both initial and continuation therapy when the patient meets the applicable clinical criteria. Requests for dosing outside the established 60 mg/kg IV once weekly regimen will be reviewed on a case-by-case basis by a clinician (Medical Director or Pharmacist).

Prior authorization required
Typical approval duration: 1 year
Doses outside policy dosing reviewed case-by-case

Step Therapy

No step therapy is required prior to authorization. However, prior authorization documentation expectations (see documentation callout) must be met at initial request and for renewals.

No step edits or trials required
Submit full prior authorization documentation with first request

Clinical Background

Alpha1-antitrypsin (AAT) deficiency is an inherited, autosomal co-dominant disorder characterized by low circulating AAT levels that increase risk for progressive emphysema, typically presenting in adulthood. Diagnosis begins with quantitative plasma AAT measurement; a serum concentration of 11 micromol/L (11 mcM) (approximately 80 mg/dL by radial immunodiffusion or <57 mg/dL by nephelometry) is considered the theoretical protective threshold and is associated with disease-causing genotypes such as ZZ, (null)(null), Z(null), and SZ. Augmentation therapy with alpha1-proteinase inhibitor aims to raise serum AAT above this threshold to slow emphysema progression and is supported for appropriately selected non-smokers or ex-smokers with airflow obstruction; it is not supported for current smokers or for COPD not due to AAT deficiency.

Key Definitions and Genotypes

Protective threshold — 11 mcM and assay equivalencies

Protective threshold (definition)Serum AAT concentration of 11 micromol/L (11 mcM) is the theoretical protective threshold

RID equivalencyApproximately 80 mg/dL if measured by radial immunodiffusion (RID)

Nephelometry equivalencyApproximately 57 mg/dL if measured by nephelometry

AAT deficiency genotypes of interest

Genotypes associated with severe deficiencyZZ genotype

Null alleles(null)(null) genotype

Compound null/Z genotypes