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摘要 重症肌无力(MG)是影响神经肌肉接头的最典型的自身免疫性疾病之一, 自身抗体以乙酰胆碱受体(AChR)复合体为靶点。绝大多数患者的眼部症状包括复视和上睑下垂, 但可能会发展为全身性疲劳性肌无力。严重的延髓肌受累可导致吞咽困难、构音障碍和呼吸困难, 进而发展为需呼吸机支持的肌无力危象。鉴于该病以眼部起病为主, 眼科医生必须掌握鉴别诊断、调查和对其的管理, 以及不断更新的治疗方法。当病情仍局限于眼外肌(眼MG)时, 近50%的患者存在抗AChR(包括簇状AChR)的IgG1和IgG3抗体。在全身性MG中, 近90%的患者存在以上情况。其他抗体包括针对肌肉特异性酪氨酸激酶(Musk)和脂蛋白受体相关蛋白4(LRP4)的抗体。尽管对重复神经刺激的反应减弱是最常见的神经生理学异常, 但对有经验的医生来说, 单纤维肌电(SFEMG)是最敏感的有助于诊断的检查。最初的治疗应使用胆碱酯酶抑制剂, 随后使用皮质类固醇和类固醇保留药物进行免疫抑制治疗。需延髓肌支持的患者可能需抢救治疗, 包括血浆置换和静脉注射免疫球蛋白(IVIg)。新的治疗靶点包括针对B淋巴细胞、补体系统、新生儿Fc受体(FcRN)和免疫系统其它靶点的治疗。 Similar content being viewed by others Introduction Myasthenia gravis (MG) is the most common autoimmune disease affecting the neuromuscular junction (NMJ) clinically characterised by fatigable weakness of the ocular, limb and bulbar (speech, swallowing and respiratory) muscles
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