Table 2.
Primary treatment options for NMO
| Medication | Dose | Route | Schedule | Monitoring | Treatment Change Considerations |
|---|---|---|---|---|---|
|
*Azathioprine (+ prednisone) |
2 - 3 mg/kg/day (+ 30 mg/day) |
Oral | 1-2 daily doses (prednisone taper after 6 – 9 months) |
Initial: TPMT activity assay. Periodic: Mean corpuscular volume (MCV) increase of at least 5 points from baseline; monthly liver function tests for first 6 months, then twice yearly; maintain absolute neutrophil counts > 1000 cells/μL. |
If MCV did not rise on initial dose, consider increase by 0.5 – 1 mg/kg/day. Or consider increasing dose or duration of prednisone. |
|
Switch to: Rituximab or mycophenolate mofetil. |
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|
| |||||
|
*Mycophenolate mofetil (+ prednisone) |
1000 – 3000 mg/day (+ 30 mg/day) |
Oral | Two daily doses (prednisone taper after 6 months) |
Absolute lymphocyte count (ALC) target of 1.0 – 1.5 k/μL; monthly liver function tests for first 6 months, then twice yearly |
If ALC goal cannot be reached at maximum dose of 3000 mg/day, observe closely for relapse. |
| Switch to: Rituximab | |||||
|
| |||||
| *Rituximab | 1000 mg for adults; 375 mg/m2 for children |
IV | Two doses of 1000 mg 14 days apart or 4 weekly doses of 375 mg/m2 for children; each pair can be given routinely q6 months without monitoring of CD19 counts, or by following CD19+ cell counts and dosing as soon as it exceeds 1%. |
Monthly CD19+ B cells starting immediately post- infusion; if CD19+ count exceeds 1% of total lymphocytes, re-dose with rituximab. If suppression of CD19+ count does not occur, consider switching to alternative. Monitor immunoglobulins yearly. |
Relapses during first 3 weeks of initial dosing are not failures. Relapses when CD19+ count is greater than 1% are failures due to undertreatment. |
|
Switch to: Azathioprine or mycophenolate mofetil. |
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|
| |||||
| *Prednisone | 15-30 mg | Oral | Daily dose; taper after 1 year |
Blood sugar to avoid hyperglycemia, blood pressure; DEXA scans as appropriate for osteoporosis; vitamin D and calcium supplementation as needed; consider proton pump inhibitors for gastric protection |
Prednisone monotherapy not recommended for long-term use beyond 1.5 years. |
|
Switch to: Azathioprine, mycophenolate or rituximab. |
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|
| |||||
| Methotrexate | 15 – 25 mg | Oral | Weekly | Check for liver toxicity every 3 months; recommend folate 1 mg supplementation; avoid non-steroidal anti- inflammatory drugs. |
Switch to: Azathioprine, mycophenolate mofetil or rituximab |
|
| |||||
| Mitoxantrone | 12 mg/m2 | IV | Monthly ×6, followed by monthly maintenance dose of 6 mg/m2. Total cumulative dose no greaterthan 120 mg/m2. |
Baseline and monthly echocardiogram to exclude patients and discontinue drug if left ventricular ejection fraction < 50%. |
Only recommended as second line agent. The maximum cumulative dose is 140 mg/m2. |
|
Switch to: Azathioprine, mycophenolate mofetil or rituximab |
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*
Recommended first-line agent