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1.1. Adults

Recommended doses for complement inhibitor as well as enzyme/protein replacement therapy FDA-approved for use in adults are summarized in Tables 1-3.  Patient profiles containing doses exceeding maximum recommendations will be reviewed.

Table 1. Adult Complement Inhibitor Maximum Dosages1-8
Drug NameTreatment IndicationDosage Form/StrengthMaximum Recommended Dosage 
avacopan (Tavneos)adjunctive treatment; severe active ANCA associated vasculitis (GPA & MPA)10 mg capsules30 mg by mouth twice daily
C1 esterase inhibitor, human (Berinert)HAE treatment500 international unit (IU) single-use vial for reconstitution20 IU/kg by IV injection as a single dose
C1 esterase inhibitor, human (Cinryze)HAE attacks, routine prevention500 unit single-use vial for reconstitution1000 U by IV infusion every 3-4 days+
C1 esterase inhibitor, human (Haegarda)HAE attacks, routine prevention2000, 3000 IU single-use vials for reconstitution60 IU/kg subcutaneously twice weekly (every 3 to 4 days)
C1 esterase inhibitor, recombinant (Ruconest)HAE treatment2100 IU/14 mL single-use vial for reconstitution
  • Greater than or equal to  84 kg:
    • 4200 units as a single IV dose; may repeat x1 if attack symptoms persist in 24-hour period
  • Less than 84 kg:
    • 50 units/kg as single IV dose up to a maximum of 4200 units per dose; may repeat x1 if attack symptoms persist in 24-hour period
pegcetacoplan (Empaveli)PNH1080 mg/ 20 mL solution for injection1080 mg subcutaneously twice weekly
ravulizumab (Ultomiris)

aHUS

PNH

245 mg/ 3.5 mL single-dose prefilled cartridgegreater than or equal to 40 kg: 490 mg once weekly (two 245 mg subcutaneous injections)

Legend

  • aHUS – atypical hemolytic uremic syndrome; ANCA- anti-neutrophil cytoplasmic autoantibody; GPA – granulomatosis with  olyangilitis; HAE – hereditary angioedema;  MPA – microscopic polyangilitis; PNH – paroxysmal nocturnal hemoglobinuria
  • + in patients not responding adequately, doses up to 2500 units (not exceeding 100 u/kg) every 3 or 4 days may be utilized based on individual patient response
  • ^ treat atypical hemolytic-uremic syndrome with ravulizumab for at least six months
Table 2. Adult Enzyme Replacement Therapy Maximum Dosages1,2,15-29
Drug NameTreatment IndicationDosage Form/StrengthMaximum Recommended Dosage
agalsidase beta (Fabrazyme)Fabry disease5 mg, 35 mg single-use vials1 mg/kg by intravenous (IV) infusion every 2 weeks
elapegademase (Revcovi)Adenosine deaminase severe combined immunodeficiency2.4 mg/1.5 mL intramuscular solution
  • treatment naïve:
    • 0.2 mg/kg as intramuscular injection twice weekly for a minimum of 12 to 24 weeks; titrate up by 0.033 mg/kg/week to maintain ADA trough Greater than 30 mmol/hr/L, trough deoxyadenosine nucleotides less than 0.02 mmol/L, and maintain immune reconstitution
  • patients transitioning from pegademase:
    • Patients receiving pegademase doses less than or equal to 30 U/kg should receive elapegademase 0.2 mg/kg intramuscularly once weekly; patients with pegademase doses Greater than 30 U/kg should receive equivalent elapegademase dose*
elosulfase alfa (Vimizim)Mucopoly-saccharidosis (MPS) IVA (Morquio A syndrome)5 mg/5 mL single-use vial2 mg/kg by IV infusion once weekly
galsulfase (Naglazyme)MPS VI (Maroteaux-Lamy syndrome)5 mg/5 mL preservative-free vials1 mg/kg by IV infusion once weekly
idursulfase (Elaprase)MPS II (Hunter syndrome)6 mg/3 mL single-use vial0.5 mg/kg as IV infusion once weekly
imiglucerase (Cerezyme)Gaucher disease, type 1 (non-neuropathic)400 mg vials for reconstitution60 U/kg by IV infusion over 1-2 hours every 2 weeks
laronidase (Aldurazyme)MPS 1 (Hurler, Hurler-Scheie forms; Scheie form with moderate to severe symptoms)2.9 mg/5 mL single-use vials0.58 mg/kg by IV infusion once weekly
migalastat (Galafold)Fabry disease with amenable galactosidase alpha gene (GLA) variant123 mg oral capsule123 mg by mouth every other day
sacrosidase (Sucraid)Congenital sucrase-isomaltase deficiency (CSID)8500 international units/mL as 118 mL oral solution bottles, 8500 units/ mL as 2 mL oral solutionGreater than 15 kg: 17,000 units orally mixed in 2-4 ounces of water or milk with each meal or snack
taliglucerase alfa (Elelyso)Gaucher disease, type 1200 unit single-use vials for reconstitution
  • treatment-naïve:
    • 60 U/kg by IV infusion once every 2 weeks
  • previously treated with imiglucerase:
    • use same unit/kg dosage for taliglucerase that was prescribed for imiglucerase and administer every two weeks
velaglucerase alfa (Vpriv)Gaucher disease, type 1400 unit single-use vials for reconstitution
  • treatment-naïve:
    • 60 U/kg as IV infusion every 2 weeks
  • previously treated with imiglucerase:
    • use same unit/kg dosage for velaglucerase that was prescribed for imiglucerase and administer every two weeks

Legend

  • * elapegademase equivalent dose to pegademase: pegademase dose (U/kg) divided by 150
Table 3. Adult Protein Replacement Therapy Maximum Dosages1,20
Drug NameTreatment IndicationDosage Form/StrengthMaximum Recommended Dosage
protein C concentrate (Ceprotin®)Severe congenital protein C deficiency (acute episode*)500 IU, 1000 IU single-use vial for reconstitution100-120 IU/kg initial dose by IV infusion, followed by 60-80 IU/kg every 6 hours for 3 doses by IV infusion
 Severe congenital protein C deficiency (short-term prophylaxis/ maintenance dose*) 45-60 IU/kg every 6 to 12 hours by IV infusion
 Severe congenital protein C deficiency (long-term prophylaxis*) 45-60 IU/kg every 12 hours by IV infusion

Legend

  • * maximum protein C concentrate infusion rate: 2 ml/min