Section 07
Myasthenia Gravis
Neuromuscular Junction Therapies
22 AGENTS
Pyridostigmine
MestinonAChE inhibitor
- Forms
- Tab: 60mg | Timespan: 180mg | Syrup: 60mg/5mL
- Dose
- 60mg q4-6h PRN
- MOA
- Inhibits acetylcholinesterase at NMJ
Neostigmine
BloxiverzAChE inhibitor
- Forms
- IV/IM/SC: 1mg/mL
- Dose
- 0.5-2mg IM/SC PRN
- MOA
- Inhibits acetylcholinesterase at NMJ
Prednisone
Deltasone / genericCorticosteroid
- Forms
- Tab: 1, 2.5, 5, 10, 20, 50mg
- Dose
- Individualized taper, often start 1mg/kg/day
- MOA
- Broad immunosuppression
Azathioprine
ImuranPurine antagonist
- Forms
- Tab: 50mg
- Dose
- Start 50mg/day, titrate to 2-3mg/kg/day
- MOA
- Inhibits lymphocyte proliferation
Mycophenolate mofetil
CellCeptPurine synthesis inhibitor
- Forms
- Tab: 500mg | Cap: 250mg
- Dose
- 1-1.5g BID
- MOA
- Inhibits lymphocyte proliferation (IMPDH)
Cyclosporine
Neoral / SandimmuneCalcineurin inhibitor
- Forms
- Cap: 25, 50, 100mg
- Dose
- 2-5mg/kg/day divided BID
- MOA
- Blocks T-cell activation (calcineurin)
Tacrolimus
PrografCalcineurin inhibitor
- Forms
- Cap: 0.5, 1, 5mg
- Dose
- 0.1mg/kg/day divided BID
- MOA
- Blocks T-cell activation (calcineurin)
Methotrexate
TrexallFolate antagonist
- Forms
- Tab: 2.5mg
- Dose
- 7.5-20mg once weekly
- MOA
- Inhibits dihydrofolate reductase
Eculizumab
SolirisComplement C5 inhibitor
- Forms
- IV: 300mg/30mL (10mg/mL)
- Dose
- Start 900mg IV weekly x4wk, then 1200mg at wk5, then 1200mg IV q2wk
- MOA
- Blocks C5 cleavage, prevents membrane attack complex
Ravulizumab
UltomirisComplement C5 inhibitor
- Forms
- IV: 300mg/30mL
- Dose
- Weight-based load (2400-3000mg) Day1, then 3000-3600mg Day15 & q8wk
- MOA
- Blocks C5 cleavage (long half-life)
Zilucoplan
ZilbrysqComplement C5 inhibitor
- Forms
- SC pen: 16.6mg/0.5mL
- Dose
- Weight-based (0.3mg/kg), once daily SC
- MOA
- Blocks C5 cleavage via macrocyclic peptide
Efgartigimod
VyvgartFcRn blocker
- Forms
- IV: 400mg/20mL | SC (Hytrulo): 1008mg
- Dose
- 10mg/kg IV weekly x4wk/cycle; next cycle per clinical response
- MOA
- Blocks neonatal Fc receptor, ↓circulating IgG
Rozanolixizumab
RystiggoFcRn blocker
- Forms
- SC: 280mg/vial, weight-based
- Dose
- Weight-based SC weekly x6 doses/cycle
- MOA
- Blocks neonatal Fc receptor, ↓circulating IgG
Rituximab
RituxanAnti-CD20 mAb
- Forms
- IV: 10mg/mL vial
- Dose
- Protocol-dependent (e.g. 375mg/m² weekly x4 or 1000mg x2)
- MOA
- Depletes CD20+ B-cells
Nipocalimab
ImaavyFcRn blocker
- Forms
- IV: 300mg/1.62mL and 1200mg/6.5mL vials
- Dose
- 30mg/kg IV once, then 15mg/kg every 2 weeks (gMG, age 12+)
- MOA
- Blocks neonatal Fc receptor, ↓circulating IgG
Immune globulin IV (IVIG)
Gamunex-C / Privigen / othersImmunomodulator
- Forms
- IV: 10% liquid (100mg/mL)
- Dose
- MG exacerbation (off-label): 2g/kg over 2-5 days | CIDP: 2g/kg load, then 1g/kg every 3 weeks
- MOA
- FcRn saturation, anti-idiotypic and complement/cytokine modulation
Immune globulin SC
HizentraImmunomodulator · SC
- Forms
- SC: 20% (200mg/mL)
- Dose
- CIDP maintenance: 0.2g/kg once weekly
- MOA
- FcRn saturation, anti-idiotypic and complement/cytokine modulation
Amifampridine
FirdapseK+ channel blocker
- Forms
- Tab: 10mg
- Dose
- LEMS: start 15-30mg/day in 3-4 doses, increase 5mg every 3-4 days; max single dose 20mg, max 80mg/day
- MOA
- Blocks presynaptic K+ channels, ↑ACh release
Cyclophosphamide
CytoxanAlkylating immunosuppressant
- Forms
- Tab: 25, 50mg | Cap: 25, 50mg | IV: 500mg, 1g, 2g vials
- Dose
- Refractory MG, NMOSD, CNS vasculitis (off-label): IV 500-1000mg/m² monthly, or oral 1-2mg/kg/day
- MOA
- DNA crosslinking; depletes proliferating lymphocytes
Eculizumab-aeeb
BkemvComplement C5 inhibitor (biosimilar)
- Forms
- IV: 300mg/30mL (10mg/mL)
- Dose
- gMG: 900mg IV weekly x4, 1200mg at week 5, then 1200mg every 2 weeks (same as Soliris); REMS
- MOA
- Blocks C5 cleavage, prevents membrane attack complex
Eculizumab-aagh
EpysqliComplement C5 inhibitor (biosimilar)
- Forms
- IV: 300mg/30mL (10mg/mL)
- Dose
- gMG: 900mg IV weekly x4, 1200mg at week 5, then 1200mg every 2 weeks (same as Soliris); REMS
- MOA
- Blocks C5 cleavage, prevents membrane attack complex
Efgartigimod alfa-hyaluronidase
Vyvgart HytruloFcRn blocker · SC
- Forms
- SC vial: 1008mg/11,200 units per 5.6mL | Prefilled syringe: 1000mg/10,000 units
- Dose
- gMG: once weekly x4 weeks per cycle | CIDP: once weekly
- MOA
- Blocks neonatal Fc receptor, ↓circulating IgG