Chang Xuting, Zhang Jie, Li Shangru, Wu Pengxia, Wang Rui, Zhang Chongfan, Wu Ye
Department of Pediatrics, Peking University First Hospital, Beijing 100034, China.
Fudan University GRADE Center, Children's Hospital of Fudan University, 210102, China.
Mult Scler Relat Disord. 2023 Apr;72:104571. doi: 10.1016/j.msard.2023.104571. Epub 2023 Feb 15.
Approximately 40% of adults and 30% of children with Myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) experience a relapsing course, but the optimal relapse prevention therapy remains unclear. A meta- analysis was conducted to investigate the efficacy of azathioprine (AZA), mycophenolate mofetil (MMF), rituximab (RTX), maintenance intravenous immunoglobulin (IVIG), and tocilizumab (TCZ) in prevention of attacks in MOGAD.
English and Chinese-language articles published from January 2010 to May 2022 were searched in PubMed, Embase, Web of Science, Cochrane, Wanfang Data, China National Knowledge Infrastructure (CNKI), and China Science and Technology Journal Database (CQVIP). Studies with fewer than three cases were excluded. Meta-analysis of the relapse-free rate, the change of annualized relapse rate (ARR)and Expanded Disability Status Scale (EDSS) scores before and after treatment, and an age subgroup analysis was performed.
A total of 41 studies were included. Three were prospective cohort studies, one was an ambispective cohort study, and 37 were retrospective cohort studies or case series. Eleven, eighteen, eighteen, eight, and two studies were included in the meta-analysis for relapse-free probability after AZA, MMF, RTX, IVIG, and TCZ therapy, respectively. The proportions of patients without relapse after AZA, MMF, RTX, IVIG, and TCZ were 65% [95% confidence interval (CI):49%-82%]), 73% (95%CI:62%-84%), 66% (95%CI:55%-77%), 79% (95%CI:66%-91%), and 93% (95%CI:54%-100%), respectively. The relapse-free rate did not significantly differ between the children and adults treated with each medication. Six, nine, ten, and three studies were included in the meta-analysis for the change of ARR before and after AZA, MMF, RTX, and IVIG therapy, respectively. ARR was significantly decreased after AZA, MMF, RTX, and IVIG therapy with a mean reduction of 1.58 (95%CI: [-2.29--0.87]), 1.32 (95%CI: [-1.57--1.07]), 1.01 (95%CI: [-1.34--0.67]), and 1.84 (95%CI: [-2.66--1.02]), respectively. The change in ARR did not significantly differ between children and adults.
AZA, MMF, RTX, maintenance IVIG, and TCZ all reduce the risk of relapse in both pediatric and adult patients with MOGAD. The literatures included in the meta-analysis were mainly retrospective studies, so large randomized prospective clinical trials are needed to compare the efficacy of different treatments.
约40%的成人和30%的儿童患有髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD），经历复发病程，但最佳的复发预防疗法仍不明确。进行了一项荟萃分析，以研究硫唑嘌呤（AZA）、霉酚酸酯（MMF）、利妥昔单抗（RTX）、维持性静脉注射免疫球蛋白（IVIG）和托珠单抗（TCZ）预防MOGAD发作的疗效。
在PubMed、Embase、科学引文索引、考克兰图书馆、万方数据、中国知网和中国科技期刊数据库中检索2010年1月至2022年5月发表的英文和中文文章。排除病例数少于3例的研究。对无复发率、治疗前后年化复发率（ARR）变化和扩展残疾状态量表（EDSS）评分进行荟萃分析，并进行年龄亚组分析。
共纳入41项研究。3项为前瞻性队列研究，1项为双向队列研究，37项为回顾性队列研究或病例系列。分别有11、18、18、8和2项研究纳入AZA、MMF、RTX、IVIG和TCZ治疗后无复发概率的荟萃分析。AZA、MMF、RTX、IVIG和TCZ治疗后无复发的患者比例分别为65%[95%置信区间（CI）：49%-82%]、73%（95%CI：62%-84%）、66%（95%CI：55%-77%）、79%（95%CI：66%-91%）和93%（95%CI：54%-100%）。每种药物治疗的儿童和成人之间无复发率无显著差异。分别有6、9、10和3项研究纳入AZA、MMF、RTX和IVIG治疗前后ARR变化的荟萃分析。AZA、MMF、RTX和IVIG治疗后ARR显著降低，平均降低分别为1.58（95%CI：[-2.29--0.87]）、1.32（95%CI：[-1.57--1.07]）、1.01（95%CI：[-1.34--0.67]）和1.84（95%CI：[-2.66--1.02]）。儿童和成人之间ARR的变化无显著差异。
AZA、MMF、RTX、维持性IVIG和TCZ均降低了儿童和成人MOGAD患者的复发风险。荟萃分析纳入的文献主要为回顾性研究，因此需要大型随机前瞻性临床试验来比较不同治疗方法的疗效。
