免疫维持治疗在儿童复发性髓鞘少突胶质细胞糖蛋白抗体相关疾病的研究进展

Research Progress in Immune Maintenance Therapy for Recurrent Oligodendrocyte Gly-coprotein Antibody Related Diseases in Chil-dren

摘要: 髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOG-IgG associated disorders, MOGAD)是一种不同于多发性硬化(multiple sclerosis, MS)和视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorder, NMOSD)的免疫介导的中枢神经系统炎性脱髓鞘疾病。该病儿童多见，且表型多样，可表现为单相或复发病程，约28%~60%的儿童患者复发，多次复发可能导致遗留视觉、认知、癫痫发作、运动障碍等神经功能障碍。控制急性发作、识别及预防复发、改善预后是临床关注的重点。目前急性期免疫治疗方案得到较大共识，而预防复发的免疫维持治疗尚未有比较公认的方案。目前治疗策略主要来自一些小样本、回顾性研究，并借助其他自身免疫性疾病的经验。本文就维持治疗的启动时机及不同方案进行综述以提高认识，为早期治疗、改善预后提供借鉴。

Abstract: Myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD) are im-mune-mediated inflammatory demyelinating diseases of the central nervous system that are dis-tinct from multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD). It is com-mon in children with a variety of phenotypes, and can manifest as a monophasic or recurrent course, with recurrence in about 28 to 60 percent of pediatric patients, and multiple recurrences may lead to neurological impairments such as residual vision, cognition, seizures, and movement disorders. Controlling acute exacerbations, identifying and preventing recurrence, and improving prognosis are the focus of clinical attention. At present, there is a great consensus on the immunotherapy regimen in the acute phase, but there is no well-established regimen for immunomaintenance therapy to prevent recurrence. Current treatment strategies are based on small, retrospective studies and experience with other autoimmune diseases. This article reviews the timing of initia-tion of maintenance therapy and different regimens to raise awareness, and provide reference for early treatment and prognosis.