RXMP-01 Alpha-1 Proteinase Inhibitors Form

Alpha-1 Proteinase Inhibitors

Updated on: 10/25/2025

Page 1 of 2 Policy Number RXMP-01 Line of Business Medicaid & Medicare Effective Date 5/22/2023 Revision Date 10/25/2025

Products: Aralast NP, Glassia, Prolastin-C, Prolastin-C Liquid, Zemaira

Override(s)
Approval Duration
Prior Authorization
Initial: 3 months Renewal: 12 months

HCPCS Code Description Drug Name Medical Benefit: Authorization Required; Pharmacy Benefit: Not covered

J0256 Injection, alpha 1-proteinase inhibitor (human), not otherwise specified, 10 mg
Aralast NP 500mg, 1000mg vial Prolastin-C 1000mg vial
Zemaira 1000mg vial
J0257 Injection, alpha 1 proteinase inhibitor (human), (Glassia), 10 mg Glassia 1000mg/50mL vial
J0256 Injection, alpha 1-proteinase inhibitor (human), not otherwise specified, 10 mg
Aralast NP 500mg, 1000mg vial Prolastin-C 1000mg vial
Zemaira 1000mg vial

Medicare Criteria Medicare Part B Coverage Criteria
Review using the most current Local Coverage Determination (LCD), National Coverage Determination (NCD), or Local Coverage Article (LCA) that applies to the Hawaii region. The LCD, NCD, or LCA can be found at: https://www.cms.gov/medicare-coverage-database/search.aspx. • No LCD/NCD/LCA found as of 10/25/2025. Review using [drug name] criteria below Medicare Part B 90-Day Transition Period For new Medicare members, a 90-day transition period applies. During this time, if a member is currently on an active course of the requested treatment, including when furnished by an out-of-network provider, Coverage and Step Therapy do not apply. After the first 90 days of enrollment, Coverage and Step Therapy Criteria must be met for continued coverage. Medicare Part B Step Therapy Criteria • For new starts, Medicare Part B Step Therapy Criteria must be met in addition to Coverage Criteria before a request may be approved.
• No step therapy

Approval Criteria Products All Products Approval Length Initial 6 months, Renewal 1 year Override Type PA, QL Initial Request Note: Pharmacy staff: For QUEST & non-ABD members with cancer, send ADRC referral with chart notes.

1. Diagnosis of congenital alpha-1 antitrypsin (AAT) deficiency (ICD-10: E88.01); AND
2. Diagnosis of emphysema (ICD-10: J43.0-J43.9); AND
3. One of the following: a. PiZZ, PiZ(null) or Pi*(null)(null) protein phenotypes (homozygous); or b. Other rare AAT disease genotypes associated with pre-treatment serum alpha1-antitrypsin (AAT) level less than 11 micromole per liter [e.g., Pi(Malton, Malton), Pi(SZ)] AND
4. One of the following:

Alpha-1 Proteinase Inhibitors

Updated on: 10/25/2025

Page 2 of 2 a. Circulating pre-treatment serum alpha1-antitrypsin (AAT) level less than 11 micromole per liter (which corresponds to less than 80 mg/dL if measured by radial immunodiffusion or less than 57 mg/dL if measured by nephelometry); or b. Member has a concomitant diagnosis of necrotizing. AND

5. Continued optimal conventional treatment for emphysema (e.g., bronchodilators); AND
6. One of the following: a. The FEV1 level is less than or equal to 65% of predicted; b. Member has experienced a rapid decline in lung function (i.e., reduction of FEV1 more than 120 mL/year) that warrants treatment; or c. Member has a concomitant diagnosis of necrotizing panniculitis
   AND
7. Member is NOT a current smoker Renewal Request Pharmacy staff: For QUEST & non-ABD members with cancer, send ADRC referral with chart notes.
8. Documentation of positive clinical response to therapy; AND

9. Continued optimal conventional treatment for emphysema (e.g., bronchodilators).

   Exclusion: Alpha-1 proteinase inhibitor therapy may not be approved for the following:

10. Individual who is IGA deficient and has IgA antibodies; OR

11. May not be approved when the above criteria are not met and for all other indication

    FDA Indications & Dosing Drug Name Dosing/Administration Indications: Alpha-1-antitrypsin deficiency Aralast (alpha1-proteinase inhibitor) 500 mg, 1000 mg vial 60 mg/kg IV infusion once weekly

    Glassia (alpha1-proteinase inhibitor) 1000 mg vial Prolastin (alpha1-proteinase inhibitor) 500 mg, 1000 mg vial Zemaira (alpha1-proteinase inhibitor) 1000 mg vial

    References

12. OptumRx, Prior Authorization Guideline, May 2023.
13. Micromedex, accessed May 2023.

14. CodeSource, accessed May 2023.

    Review History

15. 5/22/2023 HB: New (adopted from OptumRx Prior Authorization Guideline)
16. 4/2/2025 AP: Added Continuity of Care requirement