Table 1

Summary of common immunosuppressive medications used in NMO

Drug nameAuthor, yearPatients (n)Treatment regimenDuration of follow-upResults
PrednisoloneWatanabe et al639Low-dose corticosteroids 2.5–20 mg/day65 monthsMedian ARR reduced from 1.48 to 0.49; 91% of relapses occurred in the periods with 10 mg/day or less corticosteroids
AzathioprineMandler et al647Oral 2.0 mg/kg/day+Prednisolone (1 mg/kg/day) for 2 months tapering off to 75–175 mg/day Azathioprine and 10 mg on alternate days Prednisolone18 monthsAverage EDSS reduced from 8.2 to 4
Bichuetti et al6529Mean dose of 2 mg/kg28 monthsMean ARR reduced from 2.1 to 0.6
Costanzi et al6699≥2 mg/kg/day or <2 mg/kg/day22 monthsMedian ARR reduced from 2.20 to 0.52 on dose ≥2 mg/kg/day and from 2.09 to 0.82 on dose <2 mg/kg/day.
MycophenolateJacob et al6724The median dose was 2 g per day (range, 750–3000 mg per day), orally28 monthsMedian ARR reduced from 1.3 to 0.09; median EDSS improved from 6 to 5.5
RituximabCree et al 698375 mg/m2 infused once per week for 4 weeks; maintenance with 2 infusions of 1000 mg, 2 weeks apart when B cell became detectable12 monthsMedian ARR reduced from 2.5 to 0; median EDSS reduced from 7.5 to 5.5
Jacob et al 6825(1) 375 mg/m2 infused once per week for 4 weeks (n=18); (2) 1000 mg infused twice, with a 2-week interval between the infusions (n=4)19 monthsMedian ARR reduced from 1.7 (0–3.2) to 0 (0.5–5); EDSS improved from 7 to 5.
Pellkoffer et al70101g intravenously at day 1 and day 14, repeated 6–9 monthlyup to 5 infusionsARR reduced from 1.7 to 0.9
Bedi et al7123(1) Induction of four weekly intravenous 375 mg/m2 infusions followed by two infusions of the same dose biweekly every 12 months; (2) biweekly doses of 1000 mg, at 0 and six months32.5 monthsMedian ARR declined from 1.87 to 0; median EDSS from 7.0 (3–9) to 5.5 (0–8)
Kim et al7230Induction therapy (375 mg/m2 once weekly for 4 weeks or 1000 mg infused twice, with a 2-week interval between the infusions, followed by maintenance therapy (375 mg/m2, once) whenever the frequency of re-emerging CD27 memory B cells was more than 0.05% in peripheral blood mononuclear cells24 monthsThe relapse rate reduced from 2.4 (0.4–8) to 0.3 (0–4); EDSS improved from 4.4 to 3
MitoxantroneWeinstock-Guttman et al 74512 mg/m2/month for 6 months then every 3 months up to 2 years or a maximum dose of 100 mg/m224 monthsMean ARR reduced from 2.4 to 0.4.EDSS score decreased from a mean±SD of 4.40±1.88 at baseline to 2.25±0.65 at 24 months
Kim et al73206 cycles of 12 mg/m2 monthly infusions as an induction, followed by 6–12 mg/m2 every 3 months up to a maximum dose of 100–120 mg/m224 monthsARR reduced from 2.8 to 0.7; EDSS improved from 5.6–4.4
MethotrexateMinagar et al75750 mg intravenous weekly and prednisolone1 mg/kg/day49 monthsEDSS improved from 6.6 (5.0–7.0) at baseline to 5.0 (4.0–to 6.0) at 12 months and 4.5 (4–5) at 24 months
  • ARR, annualised relapse rate; EDSS, expanded disability status scale; NMO, neuromyelitis optica.