Department of Neurology, Tohoku University Graduate School of Medicine, Seiryo-machi 1-1, Aoba-ku, Sendai, Miyagi, 980-8574, Japan.
Department of Education and Support for Regional Medicine, Tohoku University Hospital, Sendai, Japan.
J Neurol. 2022 Jun;269(6):3136-3146. doi: 10.1007/s00415-021-10914-x. Epub 2021 Nov 25.
The patterns of relapse and relapse-prevention strategies for anti-myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are not completely investigated. We compared the patterns of relapse in later stages of MOGAD with those of anti-aquaporin-4 antibody (AQP4-Ab)-positive neuromyelitis optica spectrum disorder (NMOSD).
In this observational, comparative cohort study, 66 patients with MOGAD and 90 with AQP4-Ab-positive NMOSD were enrolled. We compared the patterns of relapse and annualized relapse rates (ARRs) in the first 10 years from disease onset, stratified by relapse-prevention treatments.
Approximately 50% of the patients with MOGAD experienced relapses in the first 10 years. Among those not undergoing relapse-prevention treatments, ARRs in the first 5 years were slightly lower in MOGAD patients than in AQP4-Ab-positive NMOSD patients (MOGAD vs. AQP4-Ab NMOSD: 0.19 vs. 0.30; p = 0.0753). After 5 years, the ARR decreased in MOGAD patients (MOGAD vs. AQP4-Ab NMOSD: 0.05 vs. 0.34; p = 0.0001), with a 72% reduction from the first 5 years (p = 0.0090). Eight (61.5%) of the 13 MOGAD patients with more than 10-year follow-up from disease onset showed relapse 10 years after onset. Clustering in the timing and phenotype of attacks was observed in both disease patients. The effectiveness of long-term low-dose oral PSL for relapse prevention in patients with MOGAD has not been determined.
The relapse risk in patients with MOGAD is generally lower than that in patients with AQP4-Ab-positive NMOSD, especially 5 years after onset. Meanwhile, relapses later than 10 years from onset are not rare in both diseases.
抗髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）的复发模式和预防复发策略尚未完全研究清楚。我们比较了 MOGAD 疾病后期复发模式与抗水通道蛋白 4 抗体（AQP4-Ab）阳性视神经脊髓炎谱系疾病（NMOSD）的复发模式。
在这项观察性、比较队列研究中，纳入了 66 例 MOGAD 患者和 90 例 AQP4-Ab 阳性 NMOSD 患者。我们比较了在疾病发病后的前 10 年内，根据预防复发治疗，复发模式和年化复发率（ARR）。
约 50%的 MOGAD 患者在发病后的前 10 年内经历了复发。在未进行预防复发治疗的患者中，MOGAD 患者在前 5 年的 ARR 略低于 AQP4-Ab 阳性 NMOSD 患者（MOGAD 与 AQP4-Ab NMOSD：0.19 与 0.30；p=0.0753）。5 年后，MOGAD 患者的 ARR 下降（MOGAD 与 AQP4-Ab NMOSD：0.05 与 0.34；p=0.0001），与前 5 年相比降低了 72%（p=0.0090）。13 例 MOGAD 患者中有 8 例（61.5%）在发病后超过 10 年的随访中出现发病 10 年后的复发。两种疾病患者的攻击时间和表型都存在聚集现象。长期低剂量口服 PSL 预防 MOGAD 患者复发的有效性尚未确定。
MOGAD 患者的复发风险通常低于 AQP4-Ab 阳性 NMOSD 患者，尤其是在发病后 5 年。同时，两种疾病在发病 10 年后都有不常见的复发。
