Colorado Rocky Mountain Health Plans Uplizna Coverage Update

Coverage Criteria for Uplizna (inebilizumab-cdon)

Initial Therapy - NMOSD

Covered when ALL of the following are met for initial NMOSD therapy:

NMOSD initial criteria

clinical features: Presence of at least one NMOSD clinical syndrome (optic neuritis, acute myelitis, area postrema syndrome, acute brainstem syndrome, symptomatic narcolepsy/diencephalic syndrome, or symptomatic cerebral syndrome)
serology and exclusion: Positive serologic test for anti‑aquaporin‑4 immunoglobulin G (AQP4‑IgG) AND diagnosis of multiple sclerosis or other diagnoses have been ruled out

prior therapy requirement for NMOSD: Either history of failure of rituximab therapy OR both (history of intolerance or contraindication to rituximab AND physician attestation that the same intolerance/severe adverse event would not be expected to occur with Uplizna).

prescribing and dosing: Uplizna initiated and dosed according to the U.S. FDA labeled dosing for NMOSD; prescribed by, or in consultation with, a neurologist; and patient is not receiving Uplizna in combination with disallowed therapies for the same indication (e.g., multiple sclerosis disease‑modifying therapies, complement inhibitors, anti‑IL6 therapy, anti‑CD20 therapy).

Continuation Therapy - NMOSD

Covered when ALL of the following are met for continuation of NMOSD therapy:

NMOSD continuation: Documentation of positive clinical response to Uplizna; dosing according to the U.S. FDA labeled dosing for NMOSD; patient is not receiving Uplizna in combination with disallowed therapies for the same indication (e.g., multiple sclerosis DMTs, anti‑IL6 therapy, complement inhibitors, anti‑CD20 therapy); reauthorization limited to no more than 12 months.

Initial Therapy - IgG4-RD

Covered when ALL of the following are met for initial IgG4‑RD therapy:

IgG4‑RD diagnosis confirmation: Diagnosis of Immunoglobulin G4‑related disease (IgG4‑RD) with confirmation using the ACR/EULAR classification criteria: involvement of ≥1 organ consistent with IgG4‑RD, negative exclusion criteria, and positive inclusion criteria consistent with IgG4‑RD.

prior therapy requirement: History of failure, contraindication, or intolerance to glucocorticoids; AND either history of failure of rituximab therapy OR both history of intolerance/contraindication to rituximab AND physician attestation that the same intolerance/severe adverse event would not be expected to occur with Uplizna.

prescribing and dosing: Uplizna initiated and dosed according to the U.S. FDA labeled dosing for IgG4‑RD; prescribed by, or in consultation with, a specialist with expertise in IgG4‑RD; patient is not receiving Uplizna in combination with a disease‑modifying therapy for IgG4‑RD (e.g., rituximab); initial authorization will be for no more than 12 months.

Continuation Therapy - IgG4-RD

Covered when ALL of the following are met for continuation of IgG4‑RD therapy:

IgG4‑RD continuation: Documentation of positive clinical response to Uplizna; dosing according to the U.S. FDA labeled dosing for IgG4‑RD; prescribed by, or in consultation with, a specialist with expertise in IgG4‑RD; patient is not receiving Uplizna in combination with a disease‑modifying therapy for IgG4‑RD (e.g., rituximab); reauthorization will be for no more than 12 months.

Initial Therapy - gMG

Covered when ALL of the following are met for initial gMG therapy:

gMG general: Diagnosis of generalized myasthenia gravis (gMG); submission of medical records confirming patient has not failed prior Uplizna, documentation of antibody status (anti‑AChR or anti‑MuSK), MGFA Clinical Classification II, III, or IV at initiation, and MG‑ADL total score ≥ 5 at initiation.

antibody‑specific prior therapy: If anti‑AChR antibody positive: either failure of ≥2 immunosuppressive agents over ≥12 months OR failure of ≥1 immunosuppressive therapy plus ≥4 courses of plasmapheresis/IVIG over ≥12 months. If anti‑MuSK antibody positive: history of failure of ≥1 immunosuppressive agent over ≥12 months.

administration and exclusions: Patient is not receiving Uplizna in combination with disallowed agents for the same indication (e.g., complement inhibitors, FcRn blockers, immune globulin); Uplizna dosing per FDA label with subsequent dosing no sooner than 2 weeks after the first initial dose and every 6 months thereafter; prescribed by, or in consultation with, a neurologist; initial authorization will be for no more than 12 months.

Continuation Therapy - gMG

Covered when ALL of the following are met for continuation of gMG therapy:

gMG continuation: Patient previously treated with Uplizna; submission of medical records demonstrating improvement and/or maintenance of at least a 2‑point improvement in MG‑ADL from pre‑treatment baseline, reduction in signs and symptoms of myasthenia gravis, and maintenance, reduction, or discontinuation (not escalation) of baseline immunosuppressive therapy; patient is not receiving Uplizna in combination with disallowed agents (complement inhibitors, FcRn blockers, immune globulin); Uplizna not given sooner than 6 months from the start of the prior treatment cycle; dosing per U.S. FDA label; reauthorization will be for no more than 12 months.

gMG — Initial Therapy (alternate set)

Covered when ALL of the following are met:

gMG Initial Therapy: Patient has not failed a previous course of Uplizna; diagnosis of generalized myasthenia gravis; positive serologic test for anti‑AChR or anti‑MuSK antibodies; MGFA Clinical Classification II, III, or IV at initiation; MG‑ADL total score ≥ 5 at initiation; patient is not receiving Uplizna in combination with disallowed agents for the same indication; Uplizna is dosed according to the U.S. FDA labeled dosing for gMG; prescribed by, or in consultation with, a neurologist; initial authorization will be for no more than 12 months.

See antibody‑specific prior therapy requirements for step therapy details.

gMG — Antibody-specific prior therapy requirements

AND one of the following antibody‑specific prior therapy conditions is met:

Anti‑AChR path: If anti‑AChR antibody positive, either failure of at least two immunosuppressive agents over ≥12 months OR failure of at least one immunosuppressive agent plus four or more courses of plasmapheresis/plasma exchange and/or immune globulin over ≥12 months without symptom control.

Examples of immunosuppressive agents: azathioprine, corticosteroids, cyclosporine, methotrexate, mycophenolate.

Anti‑MuSK path: If anti‑MuSK antibody positive, history of failure of at least one immunosuppressive agent over the course of at least 12 months.

gMG — Continuation Therapy (detailed)

Covered for continuation when ALL of the following are met:

Continuation Therapy: Patient has previously been treated with Uplizna; documentation demonstrates at least a 2‑point improvement in MG‑ADL from pre‑treatment baseline and reduction in signs and symptoms of myasthenia gravis; maintenance, reduction, or discontinuation of baseline immunosuppressive therapy (add‑on or escalation of baseline IST, or additional rescue therapy compared to baseline is considered treatment failure); patient is not receiving Uplizna in combination with disallowed agents for the same indication; Uplizna is not given sooner than 6 months from the start of the previous treatment cycle; dosing per U.S. FDA label; prescribed by, or in consultation with, a neurologist; reauthorization will be for no more than 12 months.

Add‑on or escalation of baseline IST, or additional rescue therapy compared to baseline is considered treatment failure and may lead to denial.

Use of Uplizna (inebilizumab‑cdon) in combination with other immunomodulatory therapies for the same indication is excluded. Examples of disallowed concomitant therapies include multiple sclerosis disease‑modifying therapies (e.g., dimethyl fumarate, fingolimod, ocrelizumab), complement inhibitors (e.g., eculizumab, crovalimab, ravulizumab), and anti‑IL6 therapy (e.g., tocilizumab) when given for the same diagnosis. For generalized myasthenia gravis and other applicable indications, combination with complement inhibitors, FcRn blockers, or immune globulin for the same indication is also not permitted.

Uplizna must not be administered in combination with any of the following for treatment of the same indication: a complement inhibitor (e.g., eculizumab, ravulizumab, zilucoplan), an FcRn blocker (e.g., nipocalimab, rozanolixizumab, efgartigimod), or an immune globulin product (e.g., Hizentra, Gammagard) — concurrent use with these agents for the same diagnosis is explicitly prohibited.

Clinical trial evidence demonstrates benefit of inebilizumab in the anti‑AQP4 (AQP4‑IgG) seropositive NMOSD population (a 77.3% relative reduction in attacks), whereas patients who were anti‑AQP4 antibody negative had no evidence of benefit; therefore coverage is focused on AQP4‑IgG positive NMOSD.

For continuation authorizations, the policy considers escalation of baseline therapies or need for additional rescue interventions to represent treatment failure. Specifically, add‑on therapy, dose escalation of baseline immunosuppressive therapy, or initiation of additional rescue therapy compared with the pre‑Uplizna baseline will be treated as evidence of treatment failure and may lead to denial of reauthorization.

Coding and Score Thresholds

Covered HCPCS CodesHCPCSCovered

J1823

Injection, inebilizumab-cdon, 1 mg.

Relevant Diagnosis CodesICD-10

D89.84	IgG4-related disease
G36.0	Neuromyelitis optica [Devic]
G70.00	Myasthenia gravis without (acute) exacerbation
G70.01	Myasthenia gravis with (acute) exacerbation

Applicable ICD-10 diagnosis codesICD-10

G70.00	Myasthenia gravis without (acute) exacerbation (added to policy)
G70.01	Myasthenia gravis with (acute) exacerbation (added to policy)

MG-ADL — Initiation Threshold

MG-ADL initiation thresholdMyasthenia Gravis Activities of Daily Living (MG-ADL) total score ≥ 5 at initiation of therapy

Supporting clinical classificationMGFA Clinical Classification class II, III, or IV at initiation is required in gMG initial criteria

Documentation requirementSubmission of medical records confirming MG-ADL score at initiation is required for prior authorization

MG-ADL Minimum Score for Initiation

Minimum MG-ADL for initiationMG-ADL total score ≥ 5 is required to start Uplizna for generalized myasthenia gravis

Antibody and classification context

Authorization, Documentation, and Step Therapy Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for use of Uplizna (inebilizumab-cdon) for generalized myasthenia gravis (gMG). Initial and reauthorization requests are limited to no more than 12 months.

Initial and continuation authorizations ≤ 12 months
Prescribed by or in consultation with a neurologist
Dosed according to U.S. FDA labeled dosing for gMG

Documentation Required

Required Supporting Documentation — Initial gMG Authorization

For initial gMG requests, submit complete medical records (chart notes, lab results, antibody testing, MGFA class, MG‑ADL score, prior therapy history) confirming diagnosis and eligibility.

Confirmation of generalized myasthenia gravis diagnosis
Positive serologic test for anti‑AChR or anti‑MuSK antibodies

Background on Inebilizumab and Evidence Summary

Inebilizumab (Uplizna) is a humanized, afucosylated IgG1 monoclonal antibody directed against CD19. Its mechanism of action is depletion of CD19‑expressing B lymphocytes through antibody‑dependent cellular cytolysis. The agent is indicated for select patients with neuromyelitis optica spectrum disorder (NMOSD), immunoglobulin G4‑related disease (IgG4‑RD), and generalized myasthenia gravis (gMG), with clinical trials showing reductions in NMOSD attacks in AQP4‑IgG positive patients and efficacy signals in IgG4‑RD and antibody‑positive gMG populations.

Key Definitions and Clinical Scales

Inebilizumab Mechanism

Agent classCD19‑directed humanized afucosylated IgG1 monoclonal antibody

Primary actionBinds CD19 on pre‑B and mature B lymphocytes

Cellular effectLeads to antibody‑dependent cellular cytolysis of B lymphocytes

NMOSD — Definition and AQP4‑IgG Relevance

DefinitionNeuromyelitis optica spectrum disorder (NMOSD) — a demyelinating disorder characterized by optic neuritis, acute myelitis, area postrema syndrome, and related syndromes

Serologic relevanceClinical benefit of inebilizumab demonstrated primarily in patients who are AQP4‑IgG (anti‑AQP4) antibody positive

OpenPayer

Uplizna (inebilizumab-cdon) coverage

Coverage Criteria for Uplizna (inebilizumab-cdon)

Coding and Score Thresholds

Authorization, Documentation, and Step Therapy Requirements

Background on Inebilizumab and Evidence Summary

Key Definitions and Clinical Scales