重症肌无力自身抗体的研究进展
Research Advances in Autoantibodies in Myasthenia Gravis
摘要: 重症肌无力(MG)是一种由自身抗体介导的神经–肌肉接头(NMJ)信号传递障碍的自身免疫性疾病，临床表现为骨骼肌波动性无力和易疲劳性。近年来，随着诊断技术的发展，MG的发病率呈上升趋势。根据自身抗体谱的不同，MG可分为乙酰胆碱受体(AChR)抗体型、肌肉特异性受体酪氨酸激酶(MuSK)抗体型、低密度脂蛋白受体相关蛋白4 (LRP4)抗体型及血清抗体阴性型等。AChR抗体通过补体激活、加速AChR降解及阻断乙酰胆碱结合等机制致病；MuSK抗体多见于女性，常累及延髓肌；此外抗兰尼碱受体(RyR)抗体、LRP4抗体及聚集蛋白(agrin)抗体等新型抗体的发现为MG的病理机制和治疗提供了新视角。血清双阳性(DSP-MG)和双阴性(DSN-MG) MG的临床特征及治疗反应各异，部分DSN-MG患者可通过细胞学检测方法检出低亲和力抗体。本文综述了MG相关抗体的研究进展，探讨其致病机制、临床特征及治疗策略，为MG的精准诊断和治疗提供理论依据。
Abstract: Myasthenia gravis (MG) is an autoimmune disorder mediated by autoantibodies that impair neuromuscular junction (NMJ) signaling, clinically characterized by fluctuating skeletal muscle weakness and fatigue. In recent years, with advancements in diagnostic techniques, the incidence of MG has shown an increasing trend. Based on distinct autoantibody profiles, MG can be classified into acetylcholine receptor (AChR) antibody-positive, muscle-specific receptor tyrosine kinase (MuSK) antibody-positive, low-density lipoprotein receptor-related protein 4 (LRP4) antibody-positive, and seronegative types. AChR antibodies contribute to pathogenesis through complement activation, accelerated AChR degradation, and blockade of acetylcholine binding. MuSK antibodies are more common in females and often involve bulbar muscles. Furthermore, the discovery of novel antibodies such as anti-ryanodine receptor (RyR), LRP4, and agrin antibodies has provided new insights into the pathological mechanisms and treatment of MG. Clinical features and treatment responses vary between double seropositive (DSP-MG) and double seronegative (DSN-MG) MG patients, with some DSN-MG cases detectable for low-affinity antibodies via cytochemical assays. This article reviews recent advances in MG-related antibodies, discusses their pathogenic mechanisms, clinical characteristics, and treatment strategies, aiming to provide a theoretical basis for precise diagnosis and management of MG.
文章引用：黎芃伶, 吴鹏. 重症肌无力自身抗体的研究进展[J]. 临床医学进展, 2025, 15(11): 919-925. https://doi.org/10.12677/acm.2025.15113176

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