Molecular-targ eted therapy for refractory immune-mediated neuropat hies

在这项研究中，我们证明了利妥昔单抗治疗各种难治性免疫介导的神经病变的有效性和安全性。采用开放标签设计，对1例多灶性运动神经病变（MMN）患者、1例慢性炎症性脱髓鞘多神经病变（CIDP）患者、1例干燥综合征相关神经病变（SN）患者和4例抗mag IgM-MGUS神经病变（IgM-N）患者给予利妥昔单抗（375mg /m^2，每周静脉注射，持续4周）。减少MMN患者的总IVIg需求，虽然效果只是暂时的，但被认为对利妥昔单抗有反应。3例IgM-N患者临床状况改善，提示利妥昔单抗耐受性良好，是一种有发展前景的新药。所有患者均未发生不良事件。

In this study, we demonstrated the effect and safety of rituximab for various refractory immune-mediated neuropathies. Rituximab (375 mg/m^2 intravenously each week for 4 weeks) was administered in an openlabel design to one patient with multifocal motor neuropathy (MMN), one with chronic inflammatory demyelinating polyneuropathy (CIDP), one with neuropathy associated with Sjogren's syndrome (SN), and four with anti-MAG IgM-MGUS neuropathy (IgM-N). Reduction of total IVIg requirements of a patient with MMN, although the effect was only temporary, were suggested to be responded to rituximab. Improvement of clinical status in three patients with IgM-N suggeted that rituximab was well tolerated and is a promising new drug. No adverse events occurred in all patients.