Table 2.
Detailed attack data of NMOSD patients, categorized by age at disease onset.
| Available n | NMOSD | LO-NMOSD | EO-NMOSD | p-value1 | |
|---|---|---|---|---|---|
| ARR, mean (SD)2 | 211 | ||||
| Total attacks | 0.53 (0.44) | 0.51 (0.51) | 0.54 (0.40) | 0.352 | |
| Myelitis attacks | 0.36 (0.31) | 0.37 (0.28) | 0.35 (0.31) | 0.275 | |
| Optic neuritis attacks | 0.27 (0.36) | 0.35 (0.62) | 0.25 (0.26) | 0.690 | |
| Optic neuritis and myelitis attacks | 0.11 (0.07) | 0.15 (0.10) | 0.11 (0.06) | 0.240 | |
| Monophasic course, n (%) | 340 | 27 (7.9%) | 13 (12.3%) | 14 (6.0%) | 0.047 |
| Time to second attack, months (median, range) | 310 | 11.5 (1-491) | 8 (1-220) | 12.5 (1-491) | 0.104 |
| RAW3, median (IQR) | |||||
| RAW at all clinical attacks4 | 184 | 1.5 (0-3) | 3 (1.5-6) | 0.5 (0-2) | <0.001 |
| RAW5 at onset | 94 | 3.0 (2-5) | 4.0 (2.5-6.5) | 2.5 (1.5-3) | <0.001 |
| IVMP therapy6, n (%) | 12546 | ||||
| IVMP alone | 1014 (80.9%) | 208 (72.2%) | 806 (83.4%) | <0.001 | |
| IVMP with PE/IA | 240 (19.1%) | 80 (27.8%) | 160 (16.5%) | <0.001 | |
| Total dose of IVMP mg/attack, mean (SD) | 775 | 5192 (3145) | 6052 (3563) | 4961 (2984) | <0.001 |
| Apheresis therapy, n (%) | 338 | ||||
| Plasma exchange | 258 (76.3%) | 70 (72.9%) | 188 (77.7%) | 0.505 | |
| Immunoadsorption | 53 (15.6%) | 18 (18.8%) | 35 (14.5%) | 0.505 | |
| Plasma exchange + immunoadsorption | 27 (7.9%) | 8 (8.3%) | 19 (7.8%) | 0.505 | |
| Apheresis therapy cycles, mean (SD) | 287 | 6.75 (2.5) | 6.66 (2.46) | 6.79 (2.52) | 0.592 |
1Bold values indicate statistically significant results. 2Annualized attack rate (Number of total attacks divided by disease duration), symptom specific stratification. Only patients with at least 12 month of follow-up time were included. 3RAW: EDSS difference between basal EDSS before attack and EDSS ≥ 90 days after attack, if no further attack occurred. 4Median basal EDSS before attack: EO-NMOSD = 3.5 and LO-NMOSD = 3.0. 5RAW after disease onset.6Intravenous methylprednisolone and apheresis therapy (plasma exchange and/or immunoadsorption), subgroup specific for late- and early-onset. 61254 attacks from 199 (67.9%) EO-NMOSD and 107 (69.9%) LO-NMOSD patients. Due to missing data, detailed analysis of 200 attacks was not possible.
NMOSD, Neuromyelitis optica spectrum disorder; LO, late-onset; EO, early-onset; EDSS, Expanded Disability Status Scale; ARR, Annualized Attack Rate; PE, Plasmaexchange; IA,Immunoabsorption; IVMP, Intravenous methylprednisolone pulse; IQR, interquartile range; SD, standard deviation; mg, milligram; RAW, relapse associated worsening.