This policy is designed to address medical guidelines that are appropriate for the majority of individuals with a particular disease, illness, or condition. Each person's unique clinical circumstances may warrant individual consideration, based on review of applicable medical records.

Eculizumab (Soliris) 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:                                                                                                                        

• Flow cytometry demonstrates:
  • Greater than or equal to 10% PNH type III red blood cells; or
  • Greater than or equal to 50% of glycosylphosphatidylinositol-anchored proteins (GPI-AP)-deficient polymorphonuclear cells (PMNs); 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
• There is no evidence of an active meningococcal infection; 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;

Venous thrombosis 

• Deep vein thrombosis; or
• Pulmonary embolism; or
• Hepatic or portal vein thrombosis; or
• Mesenteric or splenic thrombosis; or
• Renal vein thrombosis; or
• Thrombophlebitis; or

Arterial thrombosis

• Embolic stroke; or
• Myocardial infarction; or
• Transient ischemic attack; or
• Unstable angina.

Eculizumab (Soliris) may be considered medically necessary in an initial six (6) week trial for the treatment of atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy when the following criteria are met:

• 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 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 outweigh the risk of meningococcal infection); and
• There is no evidence of an active meningococcal infection.

Continuation of eculizumab (Soliris) following an initial six (6) week trial for the treatment of aHUS is 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).

Eculizumab (Soliris) may be considered medically necessary in 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:

• Individual is positive for 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 documentation that treatment with two (2) or more immunosuppressive agents (azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil, methotrexate, chronic plasmapheresis, or tacrolimus), used alone or in combination 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.

Services that do not meet the above criteria will be considered experimental/investigational as the published peer reviewed literature does not support its efficacy or safety for any other indications.

Experimental/Investigational (E/I) services are not covered regardless of place of service.

The administration of Eculizumab (Soliris) is typically an outpatient procedure which is only eligible for coverage as an inpatient procedure in special circumstances, including, but not limited to, the presence of a co-morbid condition that would require monitoring in a more controlled environment such as the inpatient setting.

Services that do not meet the criteria of this policy will be considered experimental/investigational (E/I). A network provider can bill the member for the experimental/investigational service. The provider must give advance written notice informing the member that the service has been deemed E/I. The member must be provided with an estimate of the cost and the member must agree in writing to assume financial responsibility in advance of receiving the service. The signed agreement must be maintained in the provider’s records.

• Link to Provider Resource Center for the Medical Policy Update

01/2018, Coverage Criteria Revised for Eculizumab (Soliris®)
03/2018, Coverage Criteria Revised for Eculizumab (Soliris)

• Link to Diagnosis Codes

• Link to Table Attachment(s)

• Link to References