Table 1.
Common therapeutics in NMOSD
| Agent | Initial dose | Maintenance dose | Mode of action | Side effects |
|---|---|---|---|---|
| Methylprednisolone | 1000 mg daily for 3–5 days | N/A | Multiple | Insomnia, agitation, hypertension, hyperglycemia, ulcers |
| Plasmapheresis | 5–7 cycles | N/A | Removal of AQP4 IgG and reduction of cytokines | Coagulopathy, hemodynamic instability |
| Rituximab | 1000 mg weekly for 2 weeks or 375 mg/m2 weekly for 4 weeks | 375 mg/m2 or 1000 mg weekly for 2 weeks when CD19 count >1 % on flow cytometry | Anti-CD20, B cell depletion | Sepsis, infections (Herpes zoster, UTIs, URIs), leukopenia, transaminase elevation, PML is rare |
| Mycophenolate mofetil | 1000–2000 mg daily with concurrent prednisone (5–60 mg daily) | 1000–2000 mg daily | Inhibits inosine monophosphate dehydrogenase, impairs B and T cell synthesis | Photosensitivity, recurrent infections, headache, constipation, abdominal pain, leukopenia, PML is rare |
| Azathioprine | 2–3 mg/kg/day with concomitant prednisone (5–60 mg daily) for 6–12 months | 2–3 mg/kg/day | Thiopurine antagonist of endogenous purines in DNA and RNA, interferes with lymphocyte proliferation | Nausea, diarrhea, rash, recurrent infections, leukopenia, transaminase elevation, increased risk of lymphoma |
| Mitoxantrone | 12 mg/m2 for 3–6 months | 6–12 mg/m2 every 3 months | Causes DNA cross-linking and strand breaks, interferes with DNA repair | Nausea, transaminase elevation, leukopenia, hair loss, amenorrhea, minor infections including UTI and URI, rarely heart failure and acute leukemia |
| Methotrexate | Start with 7.5 mg weekly with upward titration and concomitant prednisone (5–60 mg daily) | 7.5–15 mg weekly with concurrent prednisone (5–10 mg daily for a least 6 months) | Folic acid antagonist | Pneumonitis, GI upset, cytopenia, hepatotoxicity |
| Cyclophosphamide | 1000 mg every 2 months with associated steroids | Same | Cytotoxic alkylating agent, inhibits mitosis | GI symptoms, hyponatremia, heart block, pancytopenia, opportunistic infections |
CD cluster of differentiation, UTI urinary tract infection, URI upper respiratory infection, PML progressive multifocal leukoencephalopathy.