Department of Neurology, Huashan hospital Fudan University, 200040 Shanghai, China.
Department of Neurology, Huashan hospital Fudan University, 200040 Shanghai, China.
Autoimmun Rev. 2021 May;20(5):102800. doi: 10.1016/j.autrev.2021.102800. Epub 2021 Mar 13.
Myasthenia gravis (MG) is a T cell-driven, B cell-mediated and autoantibody-dependent autoimmune disorder against neuromuscular junctions (NMJ). Accumulated evidence has emerged regarding the role of innate immunity in the pathogenesis of MG. In this review, we proposed two hypothesis underlying the pathological mechanism. In the context of gene predisposition, on the one hand, Toll-like receptors (TLRs) pathways were initiated by viral infection in the thymus with MG to generate chemokines and pro-inflammatory cytokines such as Type I interferon (IFN), which facilitate the thymus to function as a tertiary lymphoid organ (TLO). On the another hand, the antibodies against acetylcholine receptors (AChR) generated by thymus then activated the classical pathways on thymus and neuromuscular junction (NMJ). Futher, we also highlight the role of innate immune cells in the pathogenic response. Finally, we provide some future perspectives in developing new therapeutic approaches particularly targeting the innate immunity for MG.
重症肌无力（MG）是一种 T 细胞驱动、B 细胞介导和自身抗体依赖的自身免疫性疾病，针对神经肌肉接头（NMJ）。越来越多的证据表明先天免疫在 MG 的发病机制中起作用。在这篇综述中，我们提出了两种潜在的病理机制假说。在基因易感性的背景下，一方面，病毒感染在胸腺中引发 Toll 样受体（TLR）途径，产生趋化因子和促炎细胞因子，如 I 型干扰素（IFN），这有助于胸腺作为三级淋巴器官（TLO）发挥作用。另一方面，由胸腺产生的针对乙酰胆碱受体（AChR）的抗体随后激活了胸腺和神经肌肉接头（NMJ）的经典途径。此外，我们还强调了先天免疫细胞在致病反应中的作用。最后，我们为开发针对 MG 的先天免疫的新治疗方法提供了一些未来的展望。
