CurrentBlue Cross Blue Shield - IowaPolicy N/A

Uplizna

Defines prior authorization, coverage criteria, continuation criteria, required documentation, dosing/quantity limits, screening and monitoring, and billing code for Uplizna (inebilizumab-cdon) for Wellmark Blue Cross and Blue Shield members for FDA-approved indications (NMOSD AQP4+, IgG4-RD, gMG AChR/MuSK+).

Policy Summary

PayerBlue Cross Blue Shield - Iowa

PolicyUplizna

Policy CodePolicy N/A

Change TypeRevised (administrative)

Effective DateMar 26, 2026

Next Review Date

Key ActionSubmit required documentation for initial and continuation prior authorization requests (antibody test results, diagnosis confirmation, prior therapy history, MGFA class and MG-ADL for gMG, evidence of IgG4-RD flare, organ involvement) and chart notes demonstrating positive clinical response.

SourceLink

POLICY UPDATE CHANGES

Policy reviewed April 2026 and revised January 2026; current effective date March 26, 2026.

3Covered Indications (FDA-approved)

12 moApproval duration (gMG continuation)

6 moApproval duration (IgG4-RD initial)

Coverage Summary

Defines prior authorization for Uplizna (inebilizumab-cdon) and covers FDA-approved indications when criteria are met: NMOSD AQP4‑positive, IgG4‑Related Disease (IgG4‑RD), and generalized myasthenia gravis (gMG) AChR- or MuSK‑positive. Prior authorization with required documentation is necessary to initiate review. Quantity and dosing limits mirror FDA labeling and the policy's billing rules — initiation: 3 vials (300 mg) once then 3 vials (300 mg) two weeks later; maintenance: 3 vials (300 mg) every 6 months.

Initial Therapy Criteria

NMOSD - Initial Therapy (medically necessary)

Uplizna may be considered medically necessary for treatment of NMOSD in adults when ALL of the following criteria are met:

ALL of the following

Member is anti-aquaporin-4 (AQP4) antibody positive
Medication is being prescribed by, or in consultation with, a neurologist
Member exhibits one of the following core clinical characteristics of NMOSD
- Optic neuritis
- Acute myelitis
- Area postrema syndrome (episode of otherwise unexplained hiccups or nausea and vomiting)
- Acute brainstem syndrome
- Symptomatic narcolepsy or acute diencephalic clinical syndrome with NMOSD-typical diencephalic MRI lesions
- Symptomatic cerebral syndrome with NMOSD-typical brain lesions
Relapse history
- One or more relapses that required rescue therapy within the previous 12 months prior to initiating therapy
- Two or more relapses that required rescue therapy within the previous 24 months prior to initiating therapy
Member has had an inadequate response, intolerable adverse event, or documented contraindication to rituximab therapy AND Enspryng (satralizumab-mwge) therapy
Both rituximab and satralizumab must meet failure/intolerance/contraindication requirement
Concomitant therapy exclusions
- Member will not receive the requested drug concomitantly with complement-inhibitors (i.e., eculizumab, ravulizumab)
- Member will not receive the requested drug concomitantly with anti-CD20 therapy (i.e., rituximab)
- Member will not receive the requested drug concomitantly with IL-6 receptor antagonist (i.e., satralizumab-mwge)

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Not Medically Necessary / Exclusions

Not Medically Necessary

Uplizna is considered not medically necessary when:

Members who do not meet the criteria set forth above

For NMOSD initial therapy the policy requires that the member will not receive Uplizna concomitantly with any of the following: complement‑inhibitors (e.g., eculizumab, ravulizumab), anti‑CD20 therapy (e.g., rituximab), or IL‑6 receptor antagonists (e.g., satralizumab‑mwge).

Provider Actions

Prior Authorization

Prior authorization required with documentation

Submit required documentation for initial requests and for continuation requests to initiate prior authorization review. For initial requests include specific antibody test results and documentation confirming diagnosis; for continuation requests include chart notes demonstrating positive clinical response from baseline. Documentation should also include prior therapy history. For generalized myasthenia gravis (gMG) initial requests, include MGFA clinical classification and MG-ADL score. For IgG4-related disease (IgG4-RD) initial requests, include evidence of an IgG4-RD flare requiring glucocorticoid treatment and documentation of organ involvement or pathologic confirmation.

Specific antibody test results (e.g., AQP4 for NMOSD; AChR or MuSK for gMG)
Diagnosis confirmation (clinical/radiologic or pathologic evidence for IgG4-RD)
Prior therapy history (failures, intolerances, contraindications)
MGFA class and MG-ADL score for gMG
Evidence of IgG4-RD flare requiring glucocorticoid treatment and organ involvement

Documentation Required

Required testing before initiation and during therapy

Prior to initiation and during therapy, perform hepatitis B virus (HBV) screening, tuberculosis (TB) screening, and quantitative serum immunoglobulin testing. Ensure immunizations per guidelines; administer live or live-attenuated vaccines at least 4 weeks prior to initiating therapy.

Billing Rule

Quantity limits / dosing schedule

Quantity limits and dosing schedule: Initiation dosing consists of 3 vials (300 mg) given once followed by 3 vials (300 mg) two weeks later. Maintenance dosing is 3 vials (300 mg) every 6 months. Approvals may be subject to dosing limits per FDA labeling and evidence-based guidelines. Report drug using J1823 (Injection, inebilizumab-cdon, 1 mg).

J1823 - Injection, inebilizumab-cdon, 1 mg
Initiation: 3 vials (300 mg) once, then 3 vials (300 mg) two weeks later
Maintenance: 3 vials (300 mg) every 6 months
Approvals subject to FDA labeling/guidelines

Denial Risk

Denial risk if criteria not met

Claims may be denied if the member does not meet required indication-specific criteria or documentation. Common reasons include antibody negativity for the relevant indication (e.g., AQP4 negative for NMOSD, AChR/MuSK negative for gMG), lack of required prior therapy failures or documented contraindications/intolerance, missing required clinical scores or history (e.g., MGFA class, MG-ADL, relapse history), or use of concomitant prohibited therapies.

Antibody negativity (fails antibody positivity requirement)
Unmet prior therapy failure/intolerance/contraindication requirements
Missing clinical scores or documentation of relapse history
Concomitant prohibited therapies (e.g., complement inhibitors, anti-CD20, IL-6 receptor antagonists)

Clinical Evidence

Inebilizumab for IgG4-Related Disease, N Engl J Med. 2025 Mar 27IgG4-RD trial

Policy aligns patient selection with FDA labeling, clinical guidelines, and key studies; requires pre‑screening with HBV screening, TB screening, and quantitative serum immunoglobulin testing prior to initiation and recommends completing immunizations per guidelines at least 4 weeks before starting therapy. Dosing and quantity limits are consistent with FDA labeling and the policy billing rule: initiation dosing of 3 vials (300 mg) once then 3 vials (300 mg) two weeks later and maintenance dosing of 3 vials (300 mg) every 6 months. Prior authorization with documentation (antibody tests, diagnostic confirmation, prior therapy history, and clinical scores where applicable) is required.

Policy Summary

PayerBlue Cross Blue Shield - Iowa

PolicyUplizna

Policy CodePolicy N/A

Change TypeRevised (administrative)

Effective DateMar 26, 2026

Next Review Date

SourceLink

On This Page

OpenPayer

Uplizna

Coverage Summary

Initial Therapy Criteria

Continuation Therapy Criteria

Not Medically Necessary / Exclusions

Applicable Codes

Provider Actions

Clinical Evidence

Definitions

Clinical Thresholds

Revision History