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Eculizumab (Soliris) and Ravulizumab (Ultomiris)

Section: Injections
Effective Date: April 01, 2020

Description

Eculizumab (Soliris®) and ravulizumab-cwvz (UltomirisTM) are recombinant humanized monoclonal antibodies that bind to complement protein C5 and inhibits its enzymatic cleavage, blocks formation of the terminal complement complex, and thus prevents red cell lysis.

Criteria

Coverage is subject to the specific terms of the member’s benefit plan.

Federal Employee Program members (FEP) should check with their Retail Pharmacy Program to determine if prior approval is required by calling the Retail Pharmacy Program at 1-800-624-5060 (TTY: 1-800-624-5077). FEP members can also obtain the list through the www.fepblue.org website.

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Eculizumab (Soliris) or ravulizumab-cwvz (Ultomiris) may be considered medically necessary for the treatment of an individual 18 years of age or older with documented PNH to reduce hemolysis at initiation of therapy when the following criteria are met:

  • There is no evidence of an active meningococcal infection; and
  • Individual has been immunized with a meningococcal vaccine at least two (2) weeks prior to administration of the first dose of eculizumab (Soliris) (unless the clinical record documents that the risks of delaying eculizumab (Soliris) outweigh the risk of meningococcal infection); and
  • Flow cytometry demonstrates:
    • Greater than or equal to 5% PNH type III red blood cells; or
    • Greater than or equal to 50% of glycosylphosphatidylinositol-anchored proteins (GPI-AP)-deficient polymorphonuclear cells (PMNs); and
  • Hemoglobin that is less than or equal to 7 g/dL, or the individual has symptoms of anemia and the hemoglobin is less than or equal to 9 g/dL; or
  • Evidence of clinically elevated hemolysis lactate dehydrogenase (LDH) greater than or equal to 1.5 times the upper limit of normal (ULN); or
  • Documented history of a major adverse vascular event (MAVE) from thromboembolism that may include ANY ONE of the following:
    • Deep vein thrombosis; or
    • Pulmonary embolism; or
    • Hepatic or portal vein thrombosis; or
    • Mesenteric or splenic venus thrombosis; or
    • Renal vein thrombosis; or
    • Thrombophlebitis; or
    • Embolic stroke; or
    • Myocardial infarction; or
    • Transient ischemic attack; or
    • Unstable angina.

Eculizumab (Soliris) or ravulizumab-cwvz (Ultomiris) for any other indication not listed within this policy will be considered experimental/investigational and, therefore, not-covered. Scientific evidence does not support its efficacy or safety for any other indications.

Procedure Codes

J1300 J1303

Atypical Hemolytic Uremic Syndrome (aHUS)

Eculizumab (Soliris) or ravulizumab-cxvz (Ultomiris) may be considered medically necessary for an initial six (6) month trial for the treatment of aHUS when the following criteria are met:

  • There is no evidence of an active meningococcal infection; and
  • Individual has been immunized with a meningococcal vaccine at least two (2) weeks prior to administration of the first dose of eculizumab (Soliris) (unless the clinical record documents that the risks of delaying eculizumab (Soliris) outweigh the risk of meningococcal infection); and
  • The diagnosis of aHUS is supported by the absence of Shiga toxin-producing E. coli infection; and
  • Thrombotic thrombocytopenic purpura (TTP) has been ruled out (for example, normal ADAMTS 13 activity and no evidence of an ADAMTS 13 inhibitor), or if TTP cannot be ruled out by laboratory and clinical evaluation, a trial of plasma exchange did not result in clinical improvement; and
  • Individual has thrombotic microangiopathy (TMA) (not related to disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13 (ADAMTS13) deficiency, Shiga toxin Escherichia coli related hemolytic uremic syndrome (STEC-HUS) and genetic defect in cobalamin C metablolism) with evidence of TMA including ANY of the following lab values:
    • Low platelet count (thrombocytopenia); or
    • Microangiopathic hemolysis, thrombotic microangiopathy (breaking of red blood cells inside of blood vessels); or
    • Decreased kidney function (based on age) or requiring renal dialysis; or
  • Individual with end stage renal disease (ESRD) reliant on hemodialysis resulting from probable aHUS meeting ALL of the following criteria:
    • Individual is a transplant candidate; and
    • Individual is initiating the transplant process; and
    • Treatment is being utilized as prophylaxis to prevent another flare of aHUS upon or after transplantation.

Continuation of eculizumab (Soliris) or ravulizumab-cwvz (Ultomiris) following an initial six (6) month trial for the treatment of aHUS may be considered medically necessary when there is clinical improvement after the initial trial (for example, normalization of platelet count or laboratory evidence of reduced hemolysis).
Reauthorization will be for a period of 12 months.

Eculizumab (Soliris) or ravulizumab-cwvz (Ultomiris) for any other indication not listed within this policy will be considered experimental/investigational and, therefore, not-covered. Scientific evidence does not support its efficacy or safety for any other indications.

Procedure Codes

J1300 J1303

Myasthenia Gravis

Eculizumab (Soliris) may be considered medically necessary for an initial 26 week trial for the treatment of individuals 18 years of age or older with a diagnosis of myasthenia gravis when ALL the following criteria are met:

  • There is no evidence of an active meningococcal infection; and
  • Individual has been immunized with a meningococcal vaccine at least two (2) weeks prior to administration of the first dose of eculizumab (Soliris) (unless the clinical record documents that the risks of delaying eculizumab (Soliris) outweigh the risk of meningococcal infection); and
  • Individual is positivefor antiacetylcholine receptor (AchR) antibodies; and
  • Individual meets Myasthenia Gravis Foundation of America (MGFA) Clinical Classification Class II to IV*; and
  • Individual has a Myasthenia Gravis-Specific Activities of Daily Living scale (MG-ADL) total score of six (6) or greater at initiation*; and
  • Individual has refractory myasthenia gravis, with documentationthat treatment with two (2) or more immunosuppressive agents (azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil, methotrexate, chronic plasmapheresis, or tacrolimus), used alone or incombination for one year,was ineffective contraindicated or not tolerated.

Continuation of eculizumab (Soliris) may be medically necessary for individuals who demonstrate a clinically meaningful response regarding daily activities (greater than or equal to a three (3) point improvement in the MG-ADL from baseline) after 26 weeks of therapy.
Reauthorization will be for a period of 12 months.

Eculizumab (Soliris) will be considered experimental/investigational as the published peer reviewed literature does not support its efficacy or safety for any other indications.

Procedure Codes

J1300

Neuromyelitis Optica Spectrum Disorder (NMOSD)

Eculizumab (Soliris) may be considered medically necessary for an initial six (6) months for the treatment of an individual 18 years of age or older with Neuromyelitis Optica Spectrum Disorder (NMOSD) when the following criteria are met:

  • There is no evidence of an active meningococcal infection; and
  • Individual has been immunized with a meningococcal vaccine at least two (2) weeks prior to administration of the first dose of eculizumab (Soliris) (unless the clinical record documents that the risks of delaying eculizumab (Soliris) outweigh the risk of meningococcal infection); and
  • Individual is positive for anti-aquaporin-4 (AQP4) antibody; and
  • Individual exhibits ONE of the following core clinical characteristics of NMOSD:
    • Optic neuritis; or
    • Acute myelitis; or
    • Area postrema syndrome (episode of otherwise unexplained hiccups or nausea and vomiting); or
    • Acute brainstem syndrome; or
    • Symptomatic narcolepsy or acute diencephalic clinical syndrome with NMOSD-typical diencephalic MRI lesions; or
    • Symptomatic cerebral syndrome with NMOSD-typical brain lesions; and
  • Individual will not receive treatment with rituximab or mitoxantrone and eculizumab (Soliris) concomitantly.

Continuation of eculizumab (Soliris) may be medically necessary for individuals who demonstrate a clinically meaningful response including reduction in the number of relapses or improvement in activities of daily living etc. Reauthorization will be for a period of 12 months.

Eculizumab (Soliris) for any other indication will be considered experimental/investigational as the published peer reviewed literature does not support its efficacy or safety for any other indications.

Procedure Codes

J1300

NOTE: In addition to the above criteria, product specific dosage and/or frequency limits may apply in accordance with the U.S. Food and Drug Administration (FDA)-approved product prescribing information, national compendia, Centers for Medicare and Medicaid Services (CMS) and other peer reviewed resources or evidence-based guidelines. Blue Cross Blue Shield of North Dakota may deny, in full or in part, reimbursement for utilization that does not fall within the applicable dosage and/or frequency limits.

*See Table Attachment for additional information.

Diagnosis Codes

Covered Diagnosis Codes for J1300

D59.3 D59.5 G36.0 G70.00 G70.01

 

Covered Diagnosis Codes for J1303

D59.3 D59.5

Professional Statements and Societal Positions Guidelines

NA

Links