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抗髓鞘少突胶质细胞糖蛋白抗体相关疾病慢性期的复发活动。

Relapse activity in the chronic phase of anti-myelin-oligodendrocyte glycoprotein antibody-associated disease.

机构信息

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.

出版信息

J Neurol. 2022 Jun;269(6):3136-3146. doi: 10.1007/s00415-021-10914-x. Epub 2021 Nov 25.

Abstract

OBJECTIVE

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).

METHODS

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.

RESULTS

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.

CONCLUSIONS

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.

摘要

目的

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。

方法

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)。

结果

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。

结论

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。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/24c2/9120114/3d71927305d1/415_2021_10914_Fig1_HTML.jpg

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