EU paediatric MOG consortium consensus: Part 5-Treatment of paediatric myelin oligodendrocyte glycoprotein antibody-associated disorders
EU paediatric MOG consortium consensus: Part 5-Treatment of paediatric myelin oligodendrocyte glycoprotein antibody-associated disorders
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DOI：
10.1016/j.ejpn.2020.10.005
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发表时间：
2020-11-01
影响因子：
3.1
通讯作者：
Neuteboom, Rinze F.
中科院分区：
文献类型：
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作者：
Bruijstens, Arlette L.;Wendel, Eva-Maria;Neuteboom, Rinze F.
近年来，对髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）的不同临床表型、诊断和预后因素的认识显著增加。然而，对于急性发作和疾病复发的儿童，仍然缺乏循证治疗方案。目前使用的急性和维持治疗方案来自其他脱髓鞘中枢神经系统疾病，并且大多数是中枢特异性的。因此，儿科欧洲合作共识的这一部分试图根据主要来自回顾性研究的临床经验和证据提供急性和维持治疗的建议。在急性发作中，静脉内甲泼尼龙（IVMP）导致大多数患者的良好结局，随后可以逐渐减少口服类固醇，最长可达3个月，以通过抑制疾病活动来维持急性治疗的益处。静脉注射免疫球蛋白（IVIG）和血浆置换构成了对IVMP应答不足的二线治疗。首次复发后，应开始维持治疗，以防止进一步复发和永久性后遗症的可能性。共识组已经确定了四种一线治疗，包括利妥昔单抗（RTX）、硫唑嘌呤、吗替麦考酚酯或每月一次的IVIG。在维持治疗后进一步复发的情况下，共识小组建议使用RTX或IVIG进行治疗升级，然后将这两种药物结合起来，最终添加维持口服类固醇。许多悬而未决的问题仍然需要在进一步的国际前瞻性评估MOGAD治疗。这种国际合作对于扩大现有知识至关重要。(C)2020作者由Elsevier Ltd代表欧洲儿科神经病学学会出版。
In recent years, the understanding about the different clinical phenotypes, diagnostic and prognostic factors of myelin oligodendrocyte glycoprotein-antibody-associated disorders (MOGAD) has significantly increased. However, there is still lack of evidence-based treatment protocols for acute attacks and children with a relapsing course of the disease. Currently used acute and maintenance treatment regimens are derived from other demyelinating central nervous system diseases and are mostly centre-specific. Therefore, this part of the Paediatric European Collaborative Consensus attempts to provide recommendations for acute and maintenance therapy based on clinical experience and evidence available from mainly retrospective studies. In the acute attack, intravenous methylprednisolone (IVMP) leads to a favourable outcome in the majority of patients and can be followed by tapering of oral steroids up to a maximum of three months to maintain the benefit of acute treatment by suppressing disease activity. Intravenous immunoglobulins (IVIG) and plasmapheresis constitute second-line therapies in case of insufficient response to IVMP. After a first relapse, maintenance treatment should be started in order to prevent further relapses and the possibility of permanent sequelae. Four first-line therapies consisting of rituximab (RTX), azathioprine, mycophenolate mofetil or monthly IVIG have been identified by the consensus group. In case of further relapses despite maintenance treatment, the consensus group recommends treatment escalation with RTX or IVIG, followed by combining those two, and ultimately adding maintenance oral steroids. Many open questions remain which need to be addressed in further international prospective evaluation of MOGAD treatment. This international collaboration is essential to expand the state of current knowledge. (C) 2020 The Authors. Published by Elsevier Ltd on behalf of European Paediatric Neurology Society.