1. Target agent may be considered medically necessary when ALL of the following are met:
   1. Patient has ONE of the following:
      1. The patient has a diagnosis of generalized myasthenia gravis (gMG) AND ALL of the following:
         1. The patient has a positive serological test for anti-AChR antibodies (medical records required) AND
         2. The patient has a Myasthenia Gravis Foundation of America (MGFA) clinical classification class of II-IVb AND
         3. The patient has a MG-Activities of Daily Living total score of greater than or equal to 6 AND
         4. ONE of the following:
            1. The patient's current medications have been assessed and any medications known to exacerbate myasthenia gravis (e.g., beta blockers, procainamide, quinidine, magnesium, anti-programmed death receptor-1 monoclonal antibodies, hydroxychloroquine, aminoglycosides) have been discontinued OR
            2. Discontinuation of the offending agent is NOT clinically appropriate AND
         5. ONE of the following:
            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 convential agents used for the treatment of myasthenia gravis (i.e., corticosteroids, azathioprine, cyclosporine, mycophenolate mofetil, tacrolimus, methotrexate, cyclophosphamide) OR
            4. The patient required chronic intravenous immunoglobulin (IVIG) OR
            5. The patient required chronic plasmapheresis/plasma exchange AND
         6. ONE of the following: 
            1. Tried and had an inadequate response to ONE of the following preferred agent(s) Ultomiris (ravulizumab-cwvz), Rystiggo (rozanolixizumab-noli), Vyvgart (efgartigimod), Vyvgart Hytrulo (efgartigimod and hyaluronidase-qvfc) OR
            2. An intolerance or hypersensitivity to ONE of the following preferred agent(s) Ultomiris (ravulizumab-cwvz), Rystiggo (rozanolixizumab-noli), Vyvgart (efgartigimod), Vyvgart Hytrulo (efgartigimod and hyaluronidase-qvfc) OR
            3. An FDA labeled contraindication to ALL of the following preferred agent(s) Ultomiris (ravulizumab-cwvz), Rystiggo (rozanolixizumab-noli), Vyvgart (efgartigimod), Vyvgart Hytrulo (efgartigimod and hyaluronidase-qvfc) AND
         7. The patient will NOT be using the requested agent in combination with Rystiggo (rozanolixizumab-noli), Vyvgart (efgartigimod), Vyvgart Hytrulo (efgartigimod alfa and hyaluronidase-qvfc), Zilbrysq (zilucoplan), or Imaavy (nipocalimab-aahu) OR
      2. The patient has a diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) AND ALL of the following:
         1. The diagnosis was confirmed by flow cytometry with at least 2 independent flow cytometry reagents on at least 2 cell lineages (e.g., RBCs and WBCs) demonstrating that the patient's peripheral blood cells are deficient in glycosylphosphatidylinositol (GPI)-linked proteins (lab tests required) AND
         2. ONE of the following:
            1. The patient has ONE of the following:
               1. Tried and had an inadequate response to Ultomiris (ravulizumab-cwvz) OR
               2. An intolerance or hypersensitivity to Ultomiris (ravulizumab-cwvz) OR
            2. The patient has an FDA labeled contraindication to Ultomiris (ravulizumab-cwvz) OR
            3. The patient is currently treated with the requested agent (medical records including last date of infusion required) AND
         3. The patient will NOT be using the requested agent in combination with Empaveli (pegcetacoplan), Fabhalta (iptacopan), or Piasky (crovalimab-akkz) OR
      3. The patient has a diagnosis of atypical hemolytic uremic syndrome (aHUS) AND ALL of the following:
         1. The diagnosis was confirmed by ONE of the following: (medical records required)
            1. Genetic mutation (e.g., CFH CD46, CFI, C3, CFB, THBD, CFHR1, CFHR3, CFHR5) OR
            2. Antibodies to complement factors OR
            3. A differential diagnosis of complement-mediated HUS has been demonstrated (i.e., screening for Shiga toxin-producing E. coli [STEC] for STEC-HUS, pneumococcal culture of blood/sputum/cerebrospinal or pleural fluid for penumococcal-associated HUS, ADAMTS13 less than 10% activity for thrombotic thrombocytopenic purpura [TTP], screening for defective cobalamin metabolism AND
         2. The patient is negative for Shiga toxin-producing E. coli (STEC) AND
         3. ONE of the following:
            1. Tried and had an inadequate response to Ultomiris (ravulizumab-cwvz) OR
            2. An intolerance or hypersensitivity to Ultomiris (ravulizumab-cwvz) OR
            3. An FDA labeled contraindication to Ultomiris (ravulizumab-cwvz) OR
      4. The patient has a diagnosis of neuromyelitis optica spectrum disorder (NMOSD) AND ALL of the following:
         1. The patient is anti-aquaporin-4 (AQP4) antibody positive (lab test required) AND
         2. The diagnosis was confirmed by at least ONE of the following:
            1. Optic neuritis OR
            2. Acute myelitis OR
            3. Area postrema syndrome: episode of otherwise unexplained hiccups or nausea and vomiting OR
            4. Acute brainstem syndrome OR
            5. Symptomatic narcolepsy or acute diencephalic clinical syndrome with NMOSD-typical diencephalic MRI lesions OR
            6. Symptomatic cerebral syndrome with NMOSD-typical brain lesions AND
         3. The patient has had at least ONE discrete clinical attack of CNS symptoms AND
         4. Alternative diagnoses (e.g., multiple sclerosis, ischemic optic neuropathy) have been ruled out AND
         5. ONE of the following:
            1. The patient has ONE of the following:
               1. Tried and had an inadequate response to ONE of the following preferred agent(s) rituximab, Ultomiris (ravulizumab-cwvz), Uplizna (inebilizumab-cdon) OR 
               2. An intolerance or hypersensitivity to ONE of the following preferred agent(s) rituximab, Ultomiris (ravulizumab-cwvz), Uplizna (inebilizumab-cdon) OR
               3. AN FDA labeled contraindication to ALL of the following preferred agent(s) rituximab, Ultomiris (ravulizumab-cwvz), Uplizna (inebilizumab-cdon) OR
            2. The patient has severe disease, and it has been determined that rituximab, Ultomiris (ravulizumab-cwvz), AND Uplizna (inebilizumab-cdon) woudl NOT be clinically appropriate for the patient OR
            3. The patient is currently using the requested agent (medical records including last date of infusion required) AND 
         6. The patient will NOT be using the requested agent in combination with Enspryng (satrlizumab-mwge), Rituximab, or Uplizna (inebilizumab-cdon) AND
      5. The patient has another FDA labeled indication for the requested agent and route of administration AND
   2. If the patient has an FDA labeled indication, the ONE of the following:
      1. The patient's age is within FDA labeling for the requested indication for the requested agent OR
      2. There is support for the use of the requested agent for the patient's age for the requested indication AND
   3. The prescriber is a specialist in the area of the patient's diagnosis (e.g., neurologist, hematologist), or the prescriber has consulted with a specialist in the area of the patient's diagnosis AND
   4. The patient will NOT be using the requested agent in combination with Ultomiris (ravulizumab-cwvz), Soliris (eculizumab), Bkemv (eculizumab-aeeb), or Epysqli (eculizumab-aagh) AND
   5. The patient does NOT have any FDA labeled contraindications for the requested agent AND
   6. The requested quantity (dose) is within FDA labeled dosing for the requested indication

Initial approval length: 12 months

Renewal Criteria

Target agent may be considered medically necessary when ALL of the following are met:

1. The patient was previous approved for the requested agent through the plan's Medical Drug Review process (Note: patients not previously approved for the requested agent will required initial evaluation review) AND
2. ONE of the following:
   1. The patient has a diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) AND BOTH of the following:
      1. The patient has had improvements or stabilization with the requested agent (e.g., decreased requirement for RBC transfusions, stabilization/improvement of hemoglobin, reduction of lactate dehydrogenase [LDH]) (medical records required) AND
      2. The patient will NOT be using the requested agent in combination with Empaveli (pegcetacoplan), Fabhalta (iptacopan), or Piasky (crovalimab-akkz) OR
   2. The patient has a diagnosis of atypical hemolytic uremic syndrome (aHUS) AND the patient has had improvements or stabilization with the requested agent (e.g., improved platelet count, reduction of lactate dehydrogenase [LDH], stabilization/improvement of renal function) (medical records required) OR
   3. The patient has a diagnosis of generalized myasthenia gravis (gMG) AND BOTH of the following:
      1. The patient has had clinical benefit with the requested agent AND
      2. The patient will NOT be using the requested agent in combination with Rystiggo (rozanolixizumab-noli), Vyvgart (efgartigimod), Vyvgart Hytrulo (efgartigimod and hyaluronidase-qvfc), Zilbrysq (zilucoplan), or Imaavy (nipocalimab-aahu) OR
   4. The patient has a diagnosis of neuromyelitis optica spectrum disorder (NMOSD) AND BOTH of the following:
      1. The patient has had stabilization or improvement with the requested agent (e.g., decreased relapses, improvement or stabilization of vision or paralysis) (medical records required) AND
      2. The patient will NOT be using the requested agent in combination with Enspryng (satralizumab-mwge), rituximab, or Upliza (inebilizumab-cdon) OR
   5. The patient has a diagnosis other than PNH, aHUS, gMG, or NMOSD AND the patient has had improvement or stabilization with the requested agent (e.g., improvement or stablization of symptoms) (medical records required) AND
3. The prescriber is a specialist in the area of the patient's diagnosis (e.g., neurologist, hematologist), or the prescriber has consulted with a specialist in the area of the patient's diagnosis AND
4. The patient will NOT be using the requested agent in combination with Ultomiris (ravulizumab-cwvz), Soliris (eculizumab), Bkemv (eculizumab-aeeb), or Epysqli (eculizumab-aagh) AND
5. The patient does NOT have any FDA labeled contraindications for the requested agent AND
6. The requested quantity (dose) is within FDA labeled dosing for the requested indication

Renewal approval length: 12 months