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目的:探究血清抗低密度脂蛋白受体相关蛋白4抗体（LRP4-ab）阳性在成人重症肌无力（MG）患者中的发生情况，并总结LRP4-ab阳性MG患者临床特点及对治疗的反应和预后。方法:纳入首都医科大学宣武医院MG数据库中于2017年2月至2018年12月入库的成人MG（起病年龄≥18岁）患者共355例，采用商用放射免疫测定法检测其血清中乙酰胆碱受体抗体（AChR-ab）和骨骼肌特异性酪氨酸激酶抗体，采用基于细胞底物的实验方法检测LRP4-ab。回顾性分析其中LRP4-ab阳性MG患者的临床特点、对治疗的反应和预后，并与同期入库的LRP4-ab阴性MG患者进行比较。结果:355例成人MG患者中，有13例（3.7%）患者血清LRP4-ab阳性，且均同时AChR-ab阳性；男女性别比为2.3∶1，平均起病年龄54岁。其中5例行胸腺手术，病理证实3例为胸腺瘤，2例为胸腺增生。LRP4-ab阳性和阴性MG患者在起病症状、AChR-ab滴度、球肌无力、呼吸肌无力、胸腺瘤和病情最重时的美国重症肌无力基金会（MGFA）临床分型方面差异均无统计学意义，但LRP4-ab阳性MG患者中MGFA分型为Ⅲ型者（病情最重时）的比例明显高于阴性患者［分别为8/13和26.9%（7/26）， n P=0.08］。13例LRP4-ab阳性患者经治疗后，10例病情缓解，3例临床明显改善，与LRP4-ab阴性患者无明显差异。n 结论:本组MG患者的LRP4抗体阳性率为3.7%，LRP4抗体阳性MG的临床特点与阴性者无明显差别。“,”Objective:To detect anti-serum low-density lipoprotein receptor-related protein 4 (LRP4) antibody in adult patients with myasthenia gravis (MG), and to analyze the clinical characteristics, response to treatments and prognosis of LRP4 antibody-positive MG patients.Methods:Clinical data and blood samples were collected from 355 adult patients with MG (age of onset≥18 years) in Xuanwu Hospital, Capital Medical University from February 2017 to December 2018. All patients were tested for anti-acetylcholine receptor (AChR) antibody and anti-skeletal muscle-specific tyrosine kinase (MuSK) antibody by radioimmunoassay, and for anti-LRP4 antibody by cell immunofluorescence staining. The clinical features, response to treatment, and prognosis of LRP4-seropositive MG patients were analyzed retrospectively and were compared with LRP4-seronegative MG patients enrolled in the same period.Results:Thirteen of 355 (3.7%) patients had anti-LRP4 antibody. These patients were all seropositive for AChR antibody (so-called AChR/LRP4-MG). The male to female ratio was 2.3∶1, and the mean age at disease onset was 54 years. A total of five patients underwent thymectomy, of whom three patients had thymoma, two cases had thymus hyperplasia. There was no significant difference in clinical features between AChR/LRP4-MG and AChR-MG patients, but the former had a higher frequency of type Ⅲ (Myasthenia Gravis Foundation of America type at maximal worsening) than the latter [8/13 n vs 26.9%(7/26), n P=0.08]. With acetylcholinesterase inhibitor, corticosteroids, thymectomy, or immunosuppressant therapy, 10 of 13 AChR/LRP4-MG patients achieved remission, and three cases showed improvement of their clinical status, without significant difference compared with AChR-MG patients.n Conclusion:The frequency of LRP4 antibody was 3.7% in the MG patients, and there was no significant difference in clinical features between LRP4 antibody-positive patients and LRP4 antibody-negative patients.