Immunopathogenesis in Myasthenia Gravis and Neuromyelitis Optica.
Immunopathogenesis in Myasthenia Gravis and Neuromyelitis Optica.
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重症肌无力和视神经脊髓炎的免疫发病机制
DOI：
10.3389/fimmu.2017.01785
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发表时间：
2017
影响因子：
7.3
通讯作者：
Yan Y
中科院分区：
文献类型：
--
作者：
Wang Z;Yan Y
重症肌无力（MG）和视神经肌无力（NMO）是分别由抗乙酰胆碱受体（AChR）和水通道蛋白4（AQP 4）的体液免疫介导的外周神经肌肉接头（NMJ）和中枢神经系统（CNS）的自身免疫性通道病。这些疾病具有一些共同的特征，包括遗传易感性、环境因素、耐受性的破坏、T细胞和B细胞的协作、辅助性T细胞1（Th 1）/Th 2/Th 17/调节性T细胞的失衡、异常细胞因子和抗体分泌以及补体系统激活。然而，免疫机制的某些方面是独特的。两个靶点（AChR和AQP 4）都在外周和CNS中表达，但MG主要影响CNS外外周的NMJ，而NMO优先涉及CNS。在MG中，炎性细胞（包括B细胞和巨噬细胞）常浸润胸腺，而不在靶肌肉中，而在NMO中，炎性细胞（主要是多形核白细胞和巨噬细胞）的浸润总是在靶器官-脊髓中观察到。回顾这两种自身免疫性通道病的共同和差异特征，可能会扩大我们对这两种疾病的致病机制的理解，并有助于在未来开发适当的治疗方法。
Myasthenia gravis (MG) and neuromyelitis optica (NMO) are autoimmune channelopathies of the peripheral neuromuscular junction (NMJ) and central nervous system (CNS) that are mainly mediated by humoral immunity against the acetylcholine receptor (AChR) and aquaporin-4 (AQP4), respectively. The diseases share some common features, including genetic predispositions, environmental factors, the breakdown of tolerance, the collaboration of T cells and B cells, imbalances in T helper 1 (Th1)/Th2/Th17/regulatory T cells, aberrant cytokine and antibody secretion, and complement system activation. However, some aspects of the immune mechanisms are unique. Both targets (AChR and AQP4) are expressed in the periphery and CNS, but MG mainly affects the NMJ in the periphery outside of CNS, whereas NMO preferentially involves the CNS. Inflammatory cells, including B cells and macrophages, often infiltrate the thymus but not the target—muscle in MG, whereas the infiltration of inflammatory cells, mainly polymorphonuclear leukocytes and macrophages, in NMO, is always observed in the target organ—the spinal cord. A review of the common and discrepant characteristics of these two autoimmune channelopathies may expand our understanding of the pathogenic mechanism of both disorders and assist in the development of proper treatments in the future.