Meng Xin, Zeng Ziling, Wang Yunda, Guo Shuai, Wang Chunjuan, Wang Baojie, Guo Shougang
Department of Neurology, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, Jinan, Shandong, People's Republic of China.
Department of Neurology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, People's Republic of China.
Neuropsychiatr Dis Treat. 2022 May 3;18:953-964. doi: 10.2147/NDT.S358851. eCollection 2022.
To evaluate the efficacy and safety of low dosages of rituximab (RTX) in the treatment of MuSK-antibody-positive MG patients.
We retrospectively analyzed the data of MuSK-antibody-positive MG patients who were treated with low dosages of RTX from January 2018 to October 2021. The long-term treatment response to RTX was assessed by Myasthenia Gravis Foundation of America (MGFA) post-interventional status (PIS), Myasthenia Gravis Status and Treatment Intensity (MGSTI), dosage of steroid, MG-related activities of daily living (MG-ADL) and myasthenic muscle score (MMS) at the end of follow-up.
Clinical improvement was observed in all eight patients with follow-up for 8 to 29 months after treatment. At the last visit, complete stable remission had been achieved in one patient, pharmacologic remission in three patients, minimal manifestations status in three patients and improved in one patient based on the MGFA-PIS criteria. MGSTI level 2 or better had been reached in six (75%) patients at the last visit. The steroid dosage decreased from 60 mg at baseline to 15 mg at the last follow-up (p = 0.011). The average MG-ADL score decreased from 11 (range 7 to 15) to 0 (range 0 to 3; p = 0.011), and the MMS improved from 38.5 (range 24 to 60) to 100 (range 90 to 100; p = 0.012). These differences were all statistically significant. During RTX treatment and subsequent follow-up, 1 patient reported minor post-infusion malaise.
Low-dose RTX is effective and safe for treating anti-MuSK antibody positive MG patients. A long-term response is observed after treatment. Larger prospective studies are required to provide further evidence.
评估低剂量利妥昔单抗（RTX）治疗抗肌肉特异性激酶（MuSK）抗体阳性重症肌无力（MG）患者的疗效和安全性。
我们回顾性分析了2018年1月至2021年10月接受低剂量RTX治疗的MuSK抗体阳性MG患者的数据。通过美国重症肌无力基金会（MGFA）干预后状态（PIS）、重症肌无力状态与治疗强度（MGSTI）、类固醇剂量、MG相关日常生活活动（MG-ADL）以及随访结束时的肌无力肌肉评分（MMS）来评估RTX的长期治疗反应。
治疗后随访8至29个月，所有8例患者均有临床改善。末次随访时，根据MGFA-PIS标准，1例患者达到完全稳定缓解，3例患者达到药物缓解，3例患者达到最小表现状态，1例患者病情改善。末次随访时，6例（75%）患者达到MGSTI 2级或更好。类固醇剂量从基线时的60 mg降至末次随访时的15 mg（p = 0.011）。MG-ADL平均评分从11（范围7至15）降至0（范围0至3；p = 0.011），MMS从38.5（范围24至60）提高至100（范围90至100；p = 0.012）。这些差异均具有统计学意义。在RTX治疗及随后的随访期间，有患者报告了轻微的输注后不适。
低剂量RTX治疗抗MuSK抗体阳性MG患者有效且安全。治疗后观察到长期反应。需要更大规模的前瞻性研究来提供进一步的证据。
