儿童髓鞘少突胶质细胞糖蛋白抗体病复发因素及预防复发方案疗效的回顾性分析

目的 探讨儿童髓鞘少突胶质细胞糖蛋白（myelin oligodendrocyte glycoprotein，MOG）抗体病的临床特点、复发因素及预防复发方案的疗效。方法 回顾性分析2014年12月至2020年9月在中南大学湘雅医院小儿神经专科住院的41例儿童MOG抗体病患儿的临床资料，根据患儿是否复发分为单相病程组（n=19）、复发组（n=22），复发患儿根据是否采用预防治疗分为预防治疗组及未预防治疗组。分析各组患儿的临床特征及比较各预防方案治疗前、治疗中年复发率（annualized relapse rate，ARR）的差异。结果 41例患儿中最常见的首发表现为急性播散性脑脊髓炎（56%，23/41）。54%（22/41）患儿复发，共有57次复发事件，最常见的复发事件为视神经炎（30%，17/57）。复发组急性期皮质激素疗程不足3个月的患儿比例高于单相病程组（64% vs 32%，P < 0.05）。预防治疗组和未预防治疗组ARR差异无统计学意义（P > 0.05）。评估32例次预防治疗方案发现，使用利妥昔单抗、硫唑嘌呤的治疗中ARR较治疗前ARR降低（P < 0.05）。结论超半数儿童MOG抗体病会出现复发，多数复发患儿急性期皮质激素疗程不足3个月；利妥昔单抗、硫唑嘌呤可能减少复发风险。

Recurrence factors for myelin oligodendrocyte glycoprotein antibody disease in children and the effect of recurrence prevention regimens

Objective To study the clinical features and recurrence factors of myelin oligodendrocyte glycoprotein (MOG) antibody disease in children and the effect of recurrence prevention regimens. Methods A retrospective analysis was performed on the medical data of 41 children with MOG antibody disease who were hospitalized in the Department of Pediatric Neurology, Xiangya Hospital of Central South University, from December 2014 to September 2020. According to the presence or absence of recurrence, they were divided into a monophasic course group (n=19) and a recurrence group (n=22). According to whether preventive treatment for recurrence was given, the children with recurrence were further divided into a preventive treatment group and a non-preventive treatment group. The clinical features were analyzed for all groups, and the annualized relapse rate (ARR) was compared before and after treatment with prevention regimens. Results For these 41 children, acute disseminated encephalomyelitis was the most common initial manifestation and was observed in 23 children (56%). Of the 41 children, 22 (54%) experienced recurrence, with 57 recurrence events in total, among which optic neuritis was the most common event (17/57, 30%). The proportion of children in the recurrence group who were treated with corticosteroids for less than 3 months in the acute phase was higher than that in the monophasic course group (64% vs 32%; P < 0.05). There was no significant difference in the ARR between the preventive treatment and non-preventive treatment groups (P > 0.05). The assessment of preventive treatment regimens for 32 cases showed that the children treated with rituximab or azathioprine had a significant reduction in the ARR during treatment (P < 0.05). Conclusions More than half of the children with MOG antibody disease may experience recurrence. Most children with recurrence are treated with corticosteroids for less than 3 months in the acute phase. Rituximab and azathioprine may reduce the risk of recurrence.