MOG 抗体相关性疾病的治疗：一项国际调查的结果。
Treatment of MOG antibody associated disorders: results of an international survey.
机构信息
Department of Neurology, The Walton Centre NHS Foundation Trust, Liverpool, UK.
Department of Paediatric Neurology, Alder Hey Children's Hospital, Liverpool, UK.
出版信息
J Neurol. 2020 Dec;267(12):3565-3577. doi: 10.1007/s00415-020-10026-y. Epub 2020 Jul 4.
INTRODUCTION
While monophasic and relapsing forms of myelin oligodendrocyte glycoprotein antibody associated disorders (MOGAD) are increasingly diagnosed world-wide, consensus on management is yet to be developed.
OBJECTIVE
To survey the current global clinical practice of clinicians treating MOGAD.
METHOD
Neurologists worldwide with expertise in treating MOGAD participated in an online survey (February-April 2019).
RESULTS
Fifty-two responses were received (response rate 60.5%) from 86 invited experts, comprising adult (78.8%, 41/52) and paediatric (21.2%, 11/52) neurologists in 22 countries. All treat acute attacks with high dose corticosteroids. If recovery is incomplete, 71.2% (37/52) proceed next to plasma exchange (PE). 45.5% (5/11) of paediatric neurologists use IV immunoglobulin (IVIg) in preference to PE. Following an acute attack, 55.8% (29/52) of respondents typically continue corticosteroids for ≥ 3 months; though less commonly when treating children. After an index event, 60% (31/51) usually start steroid-sparing maintenance therapy (MT); after ≥ 2 attacks 92.3% (48/52) would start MT. Repeat MOG antibody status is used by 52.9% (27/51) to help decide on MT initiation. Commonly used first line MTs in adults are azathioprine (30.8%, 16/52), mycophenolate mofetil (25.0%, 13/52) and rituximab (17.3%, 9/52). In children, IVIg is the preferred first line MT (54.5%; 6/11). Treatment response is monitored by MRI (53.8%; 28/52), optical coherence tomography (23.1%; 12/52) and MOG antibody titres (36.5%; 19/52). Regardless of monitoring results, 25.0% (13/52) would not stop MT.
CONCLUSION
Current treatment of MOGAD is highly variable, indicating a need for consensus-based treatment guidelines, while awaiting definitive clinical trials.
简介
单相和复发性髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）的病例在全球范围内不断增加，然而，目前对于这种疾病的治疗管理尚未达成共识。
目的
调查全球治疗 MOGAD 的临床医生的当前临床实践。
方法
全球具有 MOGAD 治疗专长的神经病学家参与了一项在线调查（2019 年 2 月至 4 月）。
结果
从 86 位受邀专家中收到了 52 份回复（回复率为 60.5%），包括来自 22 个国家的成人（78.8%，41/52）和儿科（21.2%，11/52）神经病学家。所有专家均采用大剂量皮质类固醇治疗急性发作。如果恢复不完全，71.2%（37/52）的专家会选择进行血浆置换（PE）。45.5%（5/11）的儿科神经病学家更倾向于使用静脉注射免疫球蛋白（IVIg）而非 PE。急性发作后，55.8%（29/52）的受访者通常会继续使用皮质类固醇治疗≥3 个月；而治疗儿童患者时则不太常见。发作后，60%（31/51）的患者通常会开始进行类固醇维持治疗（MT）；发作≥2 次后，92.3%（48/52）的患者会开始 MT。52.9%（27/51）的患者会根据重复 MOG 抗体检测结果来决定是否开始 MT。成人患者中常用的一线 MT 包括硫唑嘌呤（30.8%，16/52）、霉酚酸酯（25.0%，13/52）和利妥昔单抗（17.3%，9/52）。儿童患者中，静脉注射免疫球蛋白是首选的一线 MT（54.5%，6/11）。MRI（53.8%，28/52）、光学相干断层扫描（23.1%，12/52）和 MOG 抗体滴度（36.5%，19/52）用于监测治疗反应。无论监测结果如何，25.0%（13/52）的患者不会停止 MT。
结论
目前 MOGAD 的治疗方法差异很大，这表明需要制定基于共识的治疗指南，同时还需要进行更具确定性的临床试验。
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