Zheng Yue-Liang, Su Gong-Zhang, Li Yan-Lin, Du Tong, Zhao Xue-Lu, Wang Cong-Cong, Liu Ying, Liu Bin, Duan Rui-Sheng, Li Xiao-Li
Department of Neurology, The First Affiliated Hospital of Shandong First Medical University and Shandong Provincial Qianfoshan Hospital, Jinan, China.
Department of Thoracic Surgery, The First Affiliated Hospital of Shandong First Medical University and Shandong Provincial Qianfoshan Hospital, Jinan, China.
Front Immunol. 2025 Apr 28;16:1545579. doi: 10.3389/fimmu.2025.1545579. eCollection 2025.
To investigate the clinical characteristics and mechanisms of muscle atrophy in myasthenia gravis (MG) patients who are double-seropositive with acetylcholine receptor (AChR) antibodies and low-density lipoprotein receptor-related protein 4 (LRP4) antibodies.
The clinical data, imaging characteristics, treatment methods, and prognosis of one case of MG with AChR/LRP4 antibodies complicated by muscle atrophy were analyzed. Literature on anti-AChR/LRP4 antibodies double-seropositive MG with muscle atrophy were reviewed.
Clinically, anti-AChR/LRP4 antibodies double-seropositive MG is rare, often onset after middle age, more common in females, frequently involving bulbar muscles, severe symptoms, poor prognosis, and unrelated to thymoma. Muscle atrophy in MG is not only seen in muscle-specific tyrosine kinase (MuSK)-MG but also in AChR-MG and seronegative MG. The mechanism of muscle atrophy may be related to genetic, immune, and nutritional factors.
探讨乙酰胆碱受体（AChR）抗体和低密度脂蛋白受体相关蛋白4（LRP4）抗体双阳性的重症肌无力（MG）患者肌肉萎缩的临床特征及机制。
分析1例合并肌肉萎缩的AChR/LRP4抗体阳性MG患者的临床资料、影像学特征、治疗方法及预后。复习抗AChR/LRP4抗体双阳性且合并肌肉萎缩的MG相关文献。
临床上，抗AChR/LRP4抗体双阳性MG较为罕见，常于中年后起病，女性多见，常累及延髓肌，症状严重，预后较差，且与胸腺瘤无关。MG中的肌肉萎缩不仅见于肌肉特异性酪氨酸激酶（MuSK）-MG，也见于AChR-MG和血清阴性MG。肌肉萎缩的机制可能与遗传、免疫和营养因素有关。
