Yang Xishuai, Zhang Wei, Chang Xueli, Li Zuopeng, Du Runquan, Guo Junhong
Department of Neurology, The First Clinical School of Shanxi Medical University, Taiyuan 030000, Shanxi, China; Department of Neurology, Changzhi city people's Hospital, Changzhi 046000, Shanxi, China.
Department of Neurology, The First Hospital of Shanxi Medical University, Taiyuan 030000, Shanxi, China.
Neurosci Lett. 2024 Jan 1;818:137561. doi: 10.1016/j.neulet.2023.137561. Epub 2023 Nov 19.
The study aims to evaluate the efficacy of low dose rituximab (RTX) in patients with muscle-specific kinase antibody positive myasthenia gravis (MuSK-MG).
This is a single-center, retrospective study. A total of 10 patients with MuSK-MG were admitted to the Department of Neurology, First Hospital, Shanxi Medical University, between April 2021 to April 2023. Of them, 9 patients had been treated with low dose RTX (500 mg every 6 month) and recruited in this study. The clinical information, including the severity before and after RTX treatment, were collected from the medical records. Clinical effectiveness was assessed by Myasthenia Gravis Foundation of America (MGFA)-postintervention status (PIS), MG-related activities of daily living (MG-ADL), Quantitative Myasthenia Gravis (QMG) scores, Myasthenia Gravis Quality of Life 15-item revised (MG-QOL15r), dosage of steroid at the end of follow up.
All nine patients showed clinical improvement at the final follow-up after low-dose RTX treatment. The mean dose of prednisolone decreased significantly from 50 mg at baseline to 18.33 mg at the last follow-up (z = -3.417, p = 0.001). The administration of low-dose RTX treatment led to significant improvements in ADL levels (Z = -2.675, P < 0.01), QMG score levels (Z = -2.371, P < 0.05) and QOL-15r levels (Z = -2.547, P < 0.01) at last visit.
Low-dose RTX is effective for treating MuSK-MG patients. Longer-term follow-up and larger-scale studies are needed to provide further evidence.
本研究旨在评估低剂量利妥昔单抗（RTX）对肌肉特异性激酶抗体阳性重症肌无力（MuSK-MG）患者的疗效。
这是一项单中心回顾性研究。2021年4月至2023年4月期间，共有10例MuSK-MG患者入住山西医科大学第一医院神经内科。其中，9例患者接受了低剂量RTX治疗（每6个月500毫克）并纳入本研究。从病历中收集临床信息，包括RTX治疗前后的严重程度。通过美国重症肌无力基金会（MGFA）干预后状态（PIS）、重症肌无力相关日常生活活动（MG-ADL）、重症肌无力定量（QMG）评分、重症肌无力生活质量15项修订版（MG-QOL15r）、随访结束时的类固醇剂量评估临床疗效。
所有9例患者在低剂量RTX治疗后的最终随访中均显示临床改善。泼尼松龙的平均剂量从基线时的50毫克显著降至最后一次随访时的18.33毫克（z = -3.417，p = 0.001）。低剂量RTX治疗导致最后一次就诊时ADL水平（Z = -2.675, P < 0.01）、QMG评分水平（Z = -2.371, P < 0.05）和QOL-15r水平（Z = -2.547, P < 0.01）显著改善。
低剂量RTX对治疗MuSK-MG患者有效。需要进行更长期的随访和更大规模的研究以提供进一步的证据。
