难治性重症肌无力患者对利妥昔单抗的反应：一项回顾性研究。
Response of patients with refractory myasthenia gravis to rituximab: a retrospective study.
机构信息
Yale University School of Medicine, Department of Neurology, Division of Neuromuscular Disorders, 15 York Street, LCI #902, New Haven, CT 06510, USA.
出版信息
Ther Adv Neurol Disord. 2011 Sep;4(5):259-66. doi: 10.1177/1756285611411503.
INTRODUCTION
Myasthenia gravis, an autoimmune disorder of neuromuscular transmission, is treated by an array of immunomodulating therapies. A variable response is observed with certain patients being medically refractory.
METHODS
We report the results of 14 refractory generalized myasthenia gravis patients (6 AChR+; 8 MuSK+) treated with rituximab.
RESULTS
Sustained clinical improvement was observed in all patients as well as a reduction of conventional immunotherapies. Prednisone dose decreased a mean of 65.1%, 85.7%, and 93.8% after cycle 1, 2, and 3 of rituximab therapy, respectively. A statistically significant reduction in plasma exchange sessions was seen after cycle 1 with all patients being off of plasma exchange after cycle 3. Acetylcholine receptor antibody titers decreased a mean of 52.1% (p = 0.0046) post-cycle 2.
CONCLUSION
Our results support the hypothesis that rituximab is beneficial and well tolerated in managing refractory myasthenia gravis and nearly doubles published cases. We propose that B-cell-directed therapies may become an attractive option and suggest pursuit of a prospective trial.
简介
重症肌无力是一种神经肌肉传递障碍的自身免疫性疾病，可通过多种免疫调节疗法进行治疗。某些患者对某些治疗方法存在医学抗性，治疗效果存在差异。
方法
我们报告了 14 例难治性全身性重症肌无力患者（6 例 AChR+；8 例 MuSK+）接受利妥昔单抗治疗的结果。
结果
所有患者均观察到持续的临床改善，同时减少了常规免疫治疗。利妥昔单抗治疗 1、2、3 个周期后，泼尼松剂量分别平均降低了 65.1%、85.7%和 93.8%。在第 1 个周期后，血浆置换次数显著减少，所有患者在第 3 个周期后均停止了血浆置换。乙酰胆碱受体抗体滴度在第 2 个周期后平均降低了 52.1%（p=0.0046）。
结论
我们的结果支持利妥昔单抗在治疗难治性重症肌无力中有益且耐受性良好的假设，并使发表的病例数量几乎翻了一番。我们提出，B 细胞靶向治疗可能成为一种有吸引力的选择，并建议进行前瞻性试验。
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