Zilbrysq- NC Standard

Restricted Product(s)

• Zilbrysq™ (zilucoplan) injection

FDA Approved Use

• For the treatment of generalized myasthenia gravis (gMG) in adult patients who are antiacetylcholine receptor (AChR) antibody positive

Criteria for Approval of Restricted Product(s)

1. The patient is 18 years of age or older; AND 
2. The patient has been diagnosed with generalized Myasthenia Gravis and meets ALL the following (medical record documentation required): 
   1. Positive serology for acetylcholine receptor (AChR) autoantibodies; AND 
   2. Myasthenia Gravis-Specific Activities of Daily Living (MG-ADL) Score of ≥ 6 at baseline; AND 
   3. The patient has a Myasthenia Gravis Foundation of America (MGFA) clinical classification class of II-IVb; AND 
   4. One of the following: 
      1. The prescriber has assessed the patient’s current medications and discontinued any medications known to exacerbate myasthenia gravis (e.g., beta blockers, procainamide, quinidine, magnesium, anti-programmed death receptor-1 monoclonal antibodies, hydroxychloroquine, aminoglycosides) OR 
      2. The prescriber has provided clinical rationale indicating that discontinuation of the offending agent is not clinically appropriate; AND 
3. The patient meets ONE of the following (medical record documentation required): 
   1. The patient has tried and had an inadequate response to at least ONE conventional agent used for the treatment of myasthenia gravis (i.e., corticosteroids, azathioprine, cyclosporine, mycophenolate mofetil, tacrolimus, methotrexate, cyclophosphamide) OR 
   2. The patient has an intolerance or hypersensitivity to ONE conventional agent used for the treatment of myasthenia gravis (i.e., corticosteroids, azathioprine, cyclosporine, mycophenolate mofetil, tacrolimus, methotrexate, cyclophosphamide) OR 
   3. The patient has an FDA labeled contraindication to ALL conventional agents used for the treatment of myasthenia gravis (i.e., corticosteroids, azathioprine, cyclosporine, mycophenolate mofetil, tacrolimus, methotrexate, cyclophosphamide); AND 
4. The patient will NOT be using the requested agent in combination with any of the following Rystiggo (rozanolixizumab-noli), Soliris (eculizumab), Ultomiris (ravulizumab-cwvz), Vyvgart (efgartigimod), Vyvgart Hytrulo (efgartigimod alfa and hyaluronidase-qvfc); AND 
5. The prescribed dose and quantity are appropriate based on intended use and FDA labeling; AND 
6. The patient is being managed by or in consultation with a specialist in the area of the patient’s diagnosis (e.g., neurologist); AND 
7. For formularies that exclude (non-formulary) the requested medication, Non-formulary Exception Criteria applies.
   
   Duration of Approval: 90 days (3 months)

Continuation Criteria

1. The patient has been previously approved for Zilbrysq with Blue Cross and Blue Shield of North Carolina (Blue Cross NC) or would have met initial criteria for approval upon the start of therapy; AND 
2. The patient has experienced clinical benefit since initiation of therapy defined by an improvement from baseline in the Myasthenia GravisSpecific Activities of Daily Living (MG-ADL) (medical record documentation required); AND 
3. The patient will NOT be using the requested agent in combination with any of the following Rystiggo (rozanolixizumab-noli), Soliris (eculizumab), Ultomiris (ravulizumab-cwvz), Vyvgart (efgartigimod), Vyvgart Hytrulo (efgartigimod alfa and hyaluronidase-qvfc); AND 
4. The prescribed dose and quantity are appropriate based on intended use and FDA labeling; AND 
5. The patient continues to be managed by or in consultation with a specialist in the area of the patient’s diagnosis (e.g., neurologist)
   
   Duration of Approval: 365 days (1 year)

Quantity Limit Exception Criteria

1. The quantity (dose) requested is for documented titration purposes at the initiation of therapy (authorization for a 90 day titration period); AND 
2. The prescribed dose cannot be achieved using a lesser quantity of a higher strength; AND 
3. The quantity (dose) requested does not exceed the maximum FDA labeled dose, when specified, or to the safest studied dose per the manufacturer’s product insert; OR 
4. If the quantity (dose) requested exceeds the maximum FDA labeled dose, when specified, or to the safest studied dose per the manufacturer’s product insert, then the prescriber must submit documentation in support of therapy with a higher dose for the intended diagnosis (submitted documentation may include medical records OR fax form which reflects medical record documentation that shows the length of time the requested dose has been used, and what other medications and doses have been tried and failed).
   
   Duration of Approval: 365 days (1 year)

References

All information referenced is from FDA package insert unless otherwise noted below.

Policy Implementation/Update Information

Criteria and treatment protocols are reviewed annually by the Blue Cross NC P&T Committee, regardless of change. This policy is reviewed in Q4 annually.

January 2024: Original utilization management policy issued.