髓鞘少突胶质细胞糖蛋白抗体相关疾病:概述

Q4 Immunology and Microbiology Clinical and Experimental Neuroimmunology Pub Date : 2023-12-19 DOI:10.1111/cen3.12771
Tatsuro Misu, Yuki Matsumoto, Kimihiko Kaneko, Toshiyuki Takahashi, Yoshiki Takai, Hirohiko Ono, Chihiro Namatame, Shuhei Nishiyama, Juichi Fujimori, Hiroshi Kuroda, Ichiro Nakashima, Kazuo Fujihara, Masashi Aoki
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摘要

近年来,越来越多的证据表明,髓鞘少突胶质细胞糖蛋白(MOG)抗体与急性炎症性脱髓鞘疾病(如急性播散性脑脊髓炎(ADEM)、视神经炎、脑干和大脑皮质脑炎)的几种表现型之间存在关联,这些疾病被称为MOG抗体相关疾病(MOGAD)。约半数病例的病程是单相的,尤其是在儿童发病的 ADEM 和视神经炎中,主要是在 MOG 抗体一过性阳性的病例中。MOGAD 的病理特征被认为是急性脱髓鞘病变,伴有 CD4 优势细胞浸润、体液免疫沉积、血管周围炎症和周围脱髓鞘,这与多发性硬化症不同。目前,MOGAD的诊断依据是2023年推出的MOGAD国际专家组标准,其诊断框架包括MOGAD特异性临床特征、MOG抗体阳性、排除其他疾病三部分。MOGAD患者的预后被认为相对较轻,但问题在于难治性复发病例。在预防方面,目前还没有获得批准的药物,但推荐使用口服减量皮质类固醇、免疫抑制剂(如硫唑嘌呤和霉酚酸莫非替酯)、利妥昔单抗和维持性静脉注射免疫球蛋白。
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Myelin oligodendrocyte glycoprotein antibody-associated disorders: An overview

In recent years, there is growing evidence of associations between antibodies against myelin oligodendrocyte glycoprotein (MOG) and several phenotypes of acute inflammatory demyelinating diseases such as acute disseminated encephalomyelitis (ADEM), optic neuritis, brainstem, and cerebral cortical encephalitis, called MOG antibody associated disorders (MOGAD). Monophasic course is known in about half of cases especially in pediatric onset ADEM and optic neuritis, mainly in cases with transient positivity of MOG antibody. Pathological features of MOGAD are considered as acute demyelinating lesions with CD4 dominant cell infiltrations, the deposition of humoral immunity, perivascular inflammation and perivenous demyelination, which is distinct from multiple sclerosis. Now the diagnosis of MOGAD is based on the international panel criteria of MOGAD launched in 2023, which the diagnostic frameworks are three parts, including MOGAD-specific clinical features, MOG antibody positivity, and the exclusion of other diseases. The prognosis of MOGAD patients is considered relatively mild, but the problem is refractory relapsing cases. For its prevention, there are no approved drugs, but oral tapering corticosteroids, immunosuppressants such as azathioprine and mycophenolic mofetil, rituximab, and the maintenance intravenous immunoglobulin are recommended, and now there are a few clinical trials of promising biological drugs already approved in other neurological disorders.

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来源期刊
Clinical and Experimental Neuroimmunology
Clinical and Experimental Neuroimmunology Immunology and Microbiology-Immunology and Microbiology (miscellaneous)
CiteScore
1.60
自引率
0.00%
发文量
52
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