Criteria
Coverage is subject to the specific terms of the member’s benefit plan.
Eculizumab (Soliris) may be considered medically necessary when an individual meets the following criteria:
atypical Hemolytic Uremic Syndrome (aHUS)
Initial Criteria
- The individual must meet criteria as outlined in prescribing information (PI) including recommendations for diagnosis and age; and
- The prescriber must be, or in consult with, a hematologist or nephrologist; and
- The individual must have a diagnosis of atypical Hemolytic Uremic Syndrome (aHUS) with ONE of the following (as evidenced with submitted documentation):
- Genetic testing confirming pathogenic mutations (e.g., CFH, CD46, CFI, C3, CFB, THBD, CFHR1, CFHR3, CFHR5); or
- Antibodies to complement factors inhibiting CFH or CFI activity; and
- The individual has signs of TMA as evidenced by ALL of the following (as evidenced by submitted documentation):
- Platelet count ≤ 150 x 109 /L; and
- Hemolysis such as an elevation in serum lactate dehydrogenase (LDH); and
- Serum creatinine above the upper limits of normal, as defined by laboratory reference range or individual requires dialysis; and
- The individual does not have TMA due to one of the following (as evidenced by submitted documentation):
- A disintegrin and metalloproteinase with thrombospondin type 1 motif, individual 13 (ADAMTS13) deficiency (<10% activity for thrombotic thrombocytopenic purpura (TTP)); and
- Shiga toxin Escherichia coli related hemolytic uremic syndrome (STEC-HUS); and
- Genetic defect in cobalamin C metabolism.
Initial Authorization: 6 months
Reauthorization Criteria
Continuation of therapy with eculizumab (Soliris) may be considered medically necessary when the following is met:
- The individual must have experienced meaningful clinical benefit since starting treatment with the requested medication, as evidenced by medical documentation (e.g., chart notes) attached to the request (subject to clinical review) including one of the following scores and symptoms:
- Normalization of platelet count, as defined by laboratory reference range; or
- Normalization of lactate dehydrogenase (LDH), as defined by laboratory reference range; or
- ≥ 25% improvement in serum creatinine from baseline or ability to discontinue dialysis.
Continuation Authorization: 12 months
Myasthenia Gravis (gMG)
Initial Criteria
- The individual must meet criteria as outlined in prescribing information (PI) including recommendations for diagnosis and age; and
- The prescriber is, or in consult with, a neurologist; and
- The individual must have a diagnosis of generalized Myasthenia Gravis; and
- The provider must submit documentation of ALL of the following:
- The individual has a Myasthenia Gravis Foundation of America (MGFA) clinical classification class of II, III, or IV; and
- The individual has a Myasthenia Gravis-specific Activities of Daily Living (MG-ADL) total score ≥ 6; and
- The individual has a positive serological test for anti-AChR antibodies (lab test must be submitted); and
- The individual has had a 90-day trial to pyridostigmine; and
- The individual has had BOTH of the following:
- A 12-month trial (total duration) of at least two (2) immunosuppressive therapies (e.g., azathioprine, cyclosporine, mycophenolate mofetil, tacrolimus, methotrexate, cyclophosphamide); and
- The individual required chronic intravenous immunoglobulin (IVIG) or chronic plasmapheresis/plasma exchange (i.e., at least every 3 months over 12 months without symptom control).
Initial Authorization: 6 months
Reauthorization Criteria
Continuation of therapy with eculizumab (Soliris) may be considered medically necessary when the following is met:
- The individual must have experienced meaningful clinical benefit since starting treatment with the requested medication, as evidenced by medical documentation (e.g., chart notes) attached to the request (subject to clinical review) including one of the following scores and symptoms:
- Decreased rate of Myasthenia Gravis exacerbations; or
- A 3-point improvement in the individual’s total MG-ADL score; or
- A 5-point improvement in quantitative MG total score.
Continuation Authorization: 12 months
Neuromyelitis Optica Spectrum Disorder (NMOSD)
- The individual must meet criteria as outlined in prescribing information (PI) including recommendations for diagnosis and age; and
- The prescriber is, or in consult with, a neurologist; and
- The individual must have a diagnosis of Neuromyelitis Optica Spectrum Disorder (NMOSD); and
- The individual has positive serologic test for anti-AQP4 antibodies; and
- Patient has a history of ≥ 1 relapses that required rescue therapy within the past 12 months; and
- Patient has an Expanded Disability Status Score (EDSS) of ≤ 6.5; and
- Patient must have ONE of the core clinical characteristics from the following:
- 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
- The individual must have had a 3-month trial with Enspryng and/or Uplizna.
Initial Authorization: 6 months
Reauthorization Criteria
Continuation of therapy with eculizumab (Soliris) may be considered medically necessary when the following is met:
- The individual must have experienced stabilization, slowing of disease progression, or improvement of the condition since starting treatment with the requested medication, as evidenced by medical documentation (e.g., chart notes) attached to the request (subject to clinical review) including:
- Reduction in relapse rate
- Reduction in symptoms (such as pain, fatigue, motor function)
Continuation Authorization: 12 months
Paroxysmal Nocturnal Hemoglobinuria (PNH)
- The individual must meet criteria as outlined in prescribing information (PI) including recommendations for diagnosis and age; and
- Must be prescribed by or in consultation with a hematologist, oncologist, or immunology specialist; and
- Must have a diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) confirmed by flow cytometry (must include at least 2 different reagents tested on at least 2 cell lineages) demonstrating that individual’s peripheral blood cells are deficient in glycosylphosphatidylinositol (GPI) – linked proteins (as evidenced by submitted documentation); and
- Must have documented have ONE of the following at least 2 weeks before starting treatment:
- A full course of meningococcal, pneumococcal, and Hib vaccines; or
- A test for antibodies against encapsulated bacteria; or
- 2 weeks of antibacterial drug prophylaxis against S. pneumoniae, N. meningitis, and H. influenzae type B if vaccines are administered less than 2 weeks prior to starting therapy; and
- One of the following criteria must be met:
- individual is transfusion-dependent; or
- individual has hemoglobin ≤ 7 g/dL or Hb ≤ 9 g/dL and individual has symptoms of thromboembolic complications (e.g., abdominal pain, shortness of breath, chest pain, end-organ damage, fatigue).
Initial Authorization: 6 months
Reauthorization Criteria
Continuation of therapy with eculizumab (Soliris) may be considered medically necessary when the following is met:
- The individual must have experienced stabilization, slowing of disease progression, or improvement of the condition since starting treatment with the requested medication, as evidenced by medical documentation (e.g., chart notes) attached to the request (subject to clinical review
- Decrease in transfusions from baseline
- Increase in hemoglobin (Hb) by ≥ 1 g/dL from baseline
- Normalization in LDH levels ≤ 280 U/L
Continuation Authorization: 12 months
Eculizumab (Soliris) 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
