Department of Neurology, Haukeland University Hospital, Norway.
Department of Clinical Medicine, University of Bergen, Bergen, Norway.
Curr Opin Immunol. 2017 Dec;49:9-13. doi: 10.1016/j.coi.2017.07.006. Epub 2017 Aug 4.
Myasthenia gravis (MG) is an autoimmune antibody-mediated disease characterized by muscle weakness and fatigability. It is believed that the initial steps triggering humoral immunity in MG take place inside thymic tissue and thymoma. The immune response against one or several epitopes expressed on thymic tissue cells spills over to neuromuscular junction components sharing the same epitope causing humoral autoimmunity and antibody production. The main cause of MG is acetylcholine receptor antibodies. However, many other neuromuscular junction membrane protein targets, intracellular and extracellular proteins are suggested to participate in MG pathophysiology. MG should be divided into subgroups based on clinical presentation and immunology. This includes onset age, clinical characteristics, thymic pathology and antibody profile. The immunological profile of these subgroups is determined by the antibodies present.
重症肌无力(MG)是一种由自身抗体介导的疾病,其特征是肌肉无力和易疲劳。据信,在 MG 中触发体液免疫的初始步骤发生在胸腺组织和胸腺瘤内。针对胸腺组织细胞上表达的一个或多个表位的免疫反应会溢出到具有相同表位的神经肌肉接头成分,导致体液自身免疫和抗体产生。MG 的主要病因是乙酰胆碱受体抗体。然而,许多其他神经肌肉接头膜蛋白靶标、细胞内和细胞外蛋白被认为参与了 MG 的病理生理学。MG 应根据临床表现和免疫学分为亚组。这包括发病年龄、临床特征、胸腺病理学和抗体谱。这些亚组的免疫学特征由存在的抗体决定。