Table 5.
Detailed data on long-term immunotherapies, categorized by age at disease onset.
| Available n | NMOSD | LO-NMOSD | EO-NMOSD | p-value1 | |
|---|---|---|---|---|---|
| Immunotherapy | |||||
| Immunotherapy (yes vs. no/unknown) | 446 | 403 (90.4%) | 134 (87.6%) | 269 (91.8%) | 0.229 |
| Switch in immunotherapy (yes vs. no/unknown) | 380 | 176 (47.3%) | 53 (41.7%) | 123 (48.6%) | 0.204 |
| Time from onset to immunotherapy, months (median, range) | 358 | 10 (0-562) | 6 (0-210) | 15 (0-562) | 0.030 |
| First line immunotherapy2 | 394 | ||||
| B-cell depletion | 172 (43.7%) | 63 (48.1%) | 109 (41.4%) | 0.210 | |
| Classical immunosuppressants | 132 (33.5%) | 43 (32.8%) | 89 (33.8%) | 0.840 | |
| IL-6-receptor inhibition | 2 (0.5%) | 0 (0%) | 2 (0.8%) | 0.317 | |
| Complement inhibition | 14 (3.6%) | 9 (6.9%) | 5 (1.9%) | 0.019 | |
| Other | 65 (16.5%) | 11 (8.4%) | 54 (20.5%) | 0.002 | |
| Immunotherapy used at the last follow-up | 390 | ||||
| B-cell depletion | 241 (61.8%) | 84 (63.6%) | 157 (60.9%) | 0.592 | |
| Classical immunosuppressants | 83 (21.3%) | 26 (19.7%) | 57 (22.1%) | 0.584 | |
| IL-6-receptor inhibition | 34 (8.7%) | 9 (6.8%) | 25 (9.7%) | 0.341 | |
| Complement inhibition | 24 (6.2%) | 13 (9.8%) | 11 (4.3%) | 0.030 | |
| Other | 8 (2.1%) | 0 (%) | 8 (3.1%) | 0.056 | |
| Post-treatment ARR3 (mean, SD) | 207 | 0.30 (0.51) | 0.23 (0.45) | 0.32 (0.53) | 0.026 |
| Attack-free under RTX first-line4 | |||||
| Attack-free after 6 mo. n, (%) | 128 | 100 (78.1%) | 35 (77.8%) | 65 (78.3%) | 0.944 |
| Attack free after 12 mo. n, (%) | 126 | 92 (73.0%) | 34 (75.6%) | 58 (71.6%) | 0.632 |
| Attack free after 36 mo. n, (%) | 86 | 49 (57.0%) | 17 (56.7%) | 32 (57.1%) | 0.966 |
| Attack free after 60 mo. n, (%) | 61 | 25 (41.0%) | 9 (42.9%) | 16 (40.0%) | 0.829 |
| Attack-free under RTX any line5 | |||||
| Attack free after 6 mo. n, (%) | 194 | 158 (81.4%) | 54 (84.4%) | 104 (80.0%) | 0.461 |
| Attack free after 12 mo. n, (%) | 185 | 143 (77.3%) | 53 (84.1%) | 90 (73.8%) | 0.111 |
| Attack free after 36 mo. n, (%) | 135 | 83 (61.5%) | 29 (64.4%) | 54 (60.0%) | 0.617 |
| Attack free after 60 mo. n, (%) | 99 | 46 (46.5%) | 14 (45.2%) | 32 (47.1%) | 0.861 |
NMOSD, Neuromyelitis optica spectrum disorder; LO, late-onset; EO, early-onset; RTX, rituximab; AZA, azathioprine; MTX, metothrexate; MMF, mycophenolate mofetil; IL-6, interleukin-6; ARR, annualized attack rate.
1Bold values indicate statistically significant results.
2Immunotherapy: B-cell depletion (RTX/inebilizumab), classical immunosuppressants (AZA, MMF, MTX, oral steroids), IL6-receptor inhibition (tocilizumab/satralizumab), Complement inhibition (eculizumab), Other (glatiramer acetate, interferon beta, mitoxantrone, fingolimod, alemtuzumab, natalizumab, dimethyl fumarate, intravenous immunoglobulins, cyclophosphamide).
3Post treatment ARR = Number of attacks after initiation of NMOSD therapy divided by the time between initiation and last follow-up.
4Frequency of clinical stable course after 6, 12, 36, and 60 month after first line therapy with RTX.
5Frequency of clinical stable course after 6, 12, 36, and 60 month after treatment during the course of the disease with RTX.