Blue Cross Blue Shield - Iowa eculizumab Coverage

Coverage Criteria

Atypical hemolytic uremic syndrome — Initial Therapy

Authorization of 6 months may be granted when ALL of the following are met:

aHUS initial criteria: ADAMTS 13 activity level above 5%ADAMTS13 >5%

Not for Shiga toxin (STEC-HUS)

aHUS exclusion: Absence of Shiga toxin

Paroxysmal nocturnal hemoglobinuria — Initial Therapy

Authorization of 6 months may be granted when ALL of the following are met:

PNH initial criteria: Diagnosis of PNH confirmed by detection of GPI-AP deficiency by flow cytometry

PNH diagnostic thresholds: GPI-AP deficiency demonstrated by either: (a) at least 5% PNH cells OR (b) at least 51% GPI-AP deficient polymorphonuclear cells

Generalized myasthenia gravis — Initial Therapy

Authorization of 6 months may be granted when ALL of the following are met:

gMG serology and severity: Anti-acetylcholine receptor (AchR) antibody positive; MGFA clinical classification II to IV; MG-ADL total score ≥6MG-ADL >=6

gMG prior therapy failure: Member has failed treatment over at least one year with at least two immunosuppressive therapies (e.g., azathioprine, cyclosporine, mycophenolate, tacrolimus, methotrexate, cyclophosphamide, rituximab), OR failed at least one immunosuppressive therapy and required chronic plasmapheresis/plasma exchange or IVIG

gMG concomitant therapy exclusion: Requested medication will not be used in combination with another complement inhibitor (e.g., Ultomiris, Zilbrysq) or neonatal Fc receptor blocker (e.g., Vyvgart, Vyvgart Hytrulo, Rystiggo)

Neuromyelitis optica spectrum disorder — Initial Therapy

Authorization of 6 months may be granted in adults when ALL of the following are met:

NMOSD serology and prescriber: Anti-aquaporin-4 (AQP4) antibody positive; medication prescribed by or in consultation with a neurologist

NMOSD core clinical characteristics: Member exhibits at least one core clinical characteristic: optic neuritis OR acute myelitis OR area postrema syndrome OR acute brainstem syndrome OR symptomatic narcolepsy/acute diencephalic syndrome with NMOSD-typical lesions OR symptomatic cerebral syndrome with NMOSD-typical lesions

NMOSD recent relapse: Member has experienced a relapse that required rescue therapy in the last 12 months prior to initiating therapyrelapse requiring rescue therapy within 12 months

NMOSD concomitant therapy exclusion:

Continuation/Reauthorization Criteria — Authorization of 12 months may be granted for continued treatment

Authorization of 12 months may be granted for continued treatment when ALL of the following are met:

aHUS continuation: No evidence of unacceptable toxicity or disease progression while on the current regimen and demonstrates a positive response to therapy (e.g., normalization of LDH and platelet counts)clinical response measures

PNH continuation: No evidence of unacceptable toxicity or disease progression while on the current regimen and demonstrates a positive response to therapy (e.g., improvement in hemoglobin, normalization of LDH)clinical response measures

gMG continuation: No evidence of unacceptable toxicity or disease progression while on the current regimen and demonstrates a positive response to therapy (e.g., improvement in MG‑ADL score, changes in QMG vs baseline)improvement in MG-ADL/QMG

Eculizumab is not indicated for the treatment of patients with Shiga toxin E. coli–related hemolytic uremic syndrome (STEC‑HUS).

The HCPCS code J1300 (Injection, eculizumab, [Soliris], 10 mg) was cancelled effective 3/31/2025 and should not be used for billing after that date.

Eculizumab is considered not medically necessary for members who do not meet the coverage criteria and reauthorization requirements described above.

Initial Therapy

Initial therapy — Initial therapy approvals of 6 months when indication-specific criteria are met.

Initial therapy approvals of 6 months when indication-specific criteria are met.

aHUS initial: ADAMTS13 activity >5% and absence of Shiga toxin

PNH initial: GPI-AP deficiency demonstrated by flow cytometry with at least 5% PNH cells or at least 51% GPI-AP deficient polymorphonuclear cells

gMG initial: AchR antibody positive; MGFA II–IV; MG‑ADL ≥6; prior immunosuppressive therapy failures as specified; not concomitant with other complement inhibitors or neonatal Fc receptor blockers

NMOSD initial: AQP4 antibody positive; neurology prescriber or consult; core clinical characteristic present; relapse requiring rescue therapy in past 12 months; not concomitant with listed immunotherapies

Continuation / Reauthorization

Reauthorization — Continuation approvals of 12 months when positive response and no unacceptable toxicity or progression.

Continuation approvals of 12 months may be granted when there is positive response to therapy and no unacceptable toxicity or disease progression.

Continuation general: No evidence of unacceptable toxicity or disease progression while on current regimen and demonstrate a positive response to therapy (examples vary by indication)

Provider Actions and Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for eculizumab (initial approvals of up to 6 months may be granted when indication‑specific criteria are met; see criteria per indication in this policy). Reauthorization approvals for NMOSD may be granted for up to 12 months when continuation criteria are met.

Initial prior authorization required for all indications; consider 6‑month initial approvals when indication criteria are satisfied.
Reauthorization for NMOSD may be granted up to 12 months when continuation criteria are met.

Documentation Required

Required Documentation

Submit the documentation listed below with initial and renewal requests. For initial requests include disease‑specific confirmation testing; for continuation requests include clinical documentation demonstrating positive response.

Atypical hemolytic uremic syndrome (aHUS): ADAMTS13 level.

Procedure and Billing Codes

Procedure/Billing CodesHCPCS

J1299	Injection, eculizumab, 2 mg (effective 4/1/2025)
J1300	Injection, eculizumab, (Soliris), 10 mg (cancelled 3/31/2025)
Q5152	Injection, eculizumab-aeeb, 2 mg (effective 4/1/2025)
Q5151	Injection, eculizumab-aagh, 2 mg (effective 4/1/2025)

Covered HCPCS/J-codes and Q-codes for eculizumab productsHCPCSCovered

J1299	Injection, eculizumab, 2 mg (effective 4/1/2025)
J1300	Injection, eculizumab, (Soliris), 10 mg (cancelled 3/31/2025)
Q5152	Injection, eculizumab-aeeb, 2 mg (effective 4/1/2025)
Q5151	Injection, eculizumab-aagh, 2 mg (effective 4/1/2025)

PNH diagnostic threshold

PNH diagnostic thresholdAt least 5% PNH cells OR at least 51% GPI-AP deficient polymorphonuclear cells (demonstrated by flow cytometry)

Test methodFlow cytometry demonstrating GPI-AP deficiency is required to confirm diagnosis

PNH cell criteria option A≥5% PNH cells

PNH cell criteria option B≥51% GPI-AP deficient polymorphonuclear cells

aHUS ADAMTS13 activity

ADAMTS13 activity thresholdADAMTS13 activity level above 5%

Step Therapy and Product Preference

Requirement	Details
Documented inadequate response or intolerable adverse event with the preferred product
Request is for Soliris for treatment of neuromyelitis optica spectrum disorder (NMOSD)
Request is for Soliris for treatment of generalized myasthenia gravis in a pediatric patient ≥6 and <18 years of age

Site of Care and Infusion Considerations

Note

Infusion center — dosing limits apply

Approvals for infusion center administration may be subject to dosing limits consistent with FDA labeling and clinical guidelines.

Billing Rule

Infusion center billing — use correct eculizumab codes

Approvals for infusion center administration may be subject to dosing limits per FDA labeling and compendia; bill using the appropriate HCPCS/J- or Q-codes for the eculizumab product administered.

Report the appropriate code for the specific eculizumab product (e.g., J1299, Q5152, Q5151).

Preferred Product and Biosimilar Guidance

Note

Preferred product — Epysqli preferred; Soliris/Bkemv non-preferred

Epysqli (eculizumab-AAGH) is the preferred eculizumab product; Soliris and Bkemv are non-preferred and coverage for non-preferred products requires clinical exception criteria to be met.

Preferred product: Epysqli (eculizumab-AAGH).
Non-preferred: Soliris, Bkemv — require documented inadequate response/intolerance or specified clinical indications.

Billing Rule

Biosimilar billing — use appropriate Q-code

Effective 4/1/2025, Q5152 and Q5151 identify specific eculizumab biosimilars; providers must use the appropriate Q-code for biosimilar products when billing.

Q5152: eculizumab-aeeb, 2 mg (effective 4/1/2025).
Q5151: eculizumab-aagh, 2 mg (effective 4/1/2025).

Definitions and Indications

FDA-Approved Indications

PNH (FDA-approved)Paroxysmal nocturnal hemoglobinuria (PNH) — to reduce hemolysis

aHUS (FDA-approved)Atypical hemolytic uremic syndrome (aHUS) — to inhibit complement-mediated thrombotic microangiopathy

gMG (FDA-approved)Generalized myasthenia gravis (gMG) in patients ≥6 years who are anti-acetylcholine receptor (AchR) antibody positive

Compendial Use

Compendial useNeuromyelitis optica spectrum disorder (NMOSD) in adult patients who are anti‑aquaporin‑4 (AQP4) antibody positive

Prescriber requirementNMOSD use described as compendial (clinical literature) rather than an FDA-approved indication

Background

Eculizumab is a monoclonal antibody complement inhibitor used to treat multiple rare, complement‑mediated disorders. FDA‑approved indications include paroxysmal nocturnal hemoglobinuria (PNH) to reduce hemolysis and atypical hemolytic uremic syndrome (aHUS) to inhibit complement‑mediated thrombotic microangiopathy; it is also approved for generalized myasthenia gravis (gMG) in patients ≥6 years who are anti‑AChR antibody positive. Compendial use includes treatment of neuromyelitis optica spectrum disorder (NMOSD) in adult patients who are anti‑AQP4 antibody positive. The policy identifies preferred and non‑preferred eculizumab products and specifies prior authorization, indication‑specific diagnostic confirmation, and continuation criteria tied to demonstrated clinical response.

OpenPayer

Eculizumab (Soliris and biosimilars) — Coverage Criteria

Coverage Criteria

Initial Therapy

Continuation / Reauthorization

Provider Actions and Requirements

Procedure and Billing Codes

Step Therapy and Product Preference

Site of Care and Infusion Considerations

Preferred Product and Biosimilar Guidance

Definitions and Indications

Background