Department of Neurology, Royal Brisbane Hospital, Butterfield Street, Herston QLD 4029, Australia.
J Neurol Neurosurg Psychiatry. 2011 Jun;82(6):659-63. doi: 10.1136/jnnp.2010.220475. Epub 2010 Nov 11.
Myasthenia gravis is an autoimmune disorder of the neuromuscular junction. Rituximab (RTX), a monoclonal antibody to CD20, leads to B lymphocyte depletion and has been used in some autoimmune disorders, including small case series of myasthenia gravis patients.
A retrospective analysis was performed of all patients with acetylcholine receptor (AChR) (11 subjects) or muscle specific kinase antibody (MuSK) positive myasthenia gravis (three subjects), who had been treated with RTX in Brisbane, Australia. In most patients 1 g of RTX, in two divided doses, was given. Patients were monitored by serial clinical assessments, flow cytometry of peripheral blood B lymphocytes and antibody testing.
RTX led to a significant improvement in symptoms in 11 of 14 patients. Doses of immunosuppressive medications were able to be reduced in 12 of 14 patients but medications could be completely ceased in only one patient. A demonstrable reduction of autoantibody levels was found in only three AChR positive patients and one MuSK positive patient, independent of clinical improvement. Peripheral blood B lymphocyte depletion was achieved in 13 out of 14 patients. B lymphocyte recovery occurred between 9 and 30 months post RTX (median 12.3 months) and was consistently associated with worsening of clinical symptoms.
Rituximab at a dose of 1 g appears to be beneficial in the treatment of patients with severe myasthenia gravis. Serial monitoring of peripheral blood B lymphocytes appears to be useful in guiding the need for further RTX therapy.
重症肌无力是一种神经肌肉接头的自身免疫性疾病。利妥昔单抗(RTX)是一种针对 CD20 的单克隆抗体,可导致 B 淋巴细胞耗竭,并已用于某些自身免疫性疾病,包括少数重症肌无力患者的病例系列研究。
对在澳大利亚布里斯班接受 RTX 治疗的乙酰胆碱受体(AChR)(11 例)或肌肉特异性激酶抗体(MuSK)阳性重症肌无力(3 例)患者进行了回顾性分析。大多数患者给予 1 g RTX,分两次给予。通过连续临床评估、外周血 B 淋巴细胞流式细胞术和抗体检测监测患者。
RTX 使 14 例患者中的 11 例症状显著改善。在 14 例患者中,12 例能够减少免疫抑制药物剂量,但只有 1 例能够完全停止使用药物。仅在 3 例 AChR 阳性患者和 1 例 MuSK 阳性患者中发现抗体水平明显降低,与临床改善无关。14 例患者中有 13 例外周血 B 淋巴细胞耗竭。B 淋巴细胞恢复发生在 RTX 后 9 至 30 个月(中位数 12.3 个月),与临床症状恶化一致。
1 g 剂量的利妥昔单抗似乎对重症肌无力患者有益。外周血 B 淋巴细胞的连续监测似乎有助于指导是否需要进一步 RTX 治疗。