PDF(Pubmed)
Abstract：
UNASSIGNED: Anti-MAG neuropathy is a slowly progressive demyelinating neuropathy that can lead to disability. The neuropathy is thought to be caused by monoclonal IgM antibodies that target the Myelin Associated Glycoprotein (MAG) in peripheral nerves. Therapy is directed at lowering the autoantibody concentrations with B-cells depleting agents, most often rituximab, based on case series and uncontrolled trials reporting improvement. There are no FDA approved treatments for anti-MAG neuropathy, however, and two relatively short duration randomized controlled trials with rituximab failed to achieve their pre-specified primary endpoints. There is also little information regarding the number or duration of treatments that are required to effectively reduce the antibody concentrations.
UNASSIGNED: We report the time course of the anti-MAG antibody response in two patients with anti-MAG neuropathy that were treated with rituximab for several years. A reduction of 50% in the anti-MAG IgM was seen after 19 and 58 months respectively, and of 70% after 74 or 104 months of treatment respectively. Titres remained low, without evidence of recurrence after the treatments were discontinued.
UNASSIGNED: Therapy of anti-MAG neuropathy with rituximab may require repeat treatments over more than one year to achieve a significant reduction in autoantibody concentrations. These considerations should inform treatment decisions and the design of clinical trials.
UNASSIGNED: We report the time course of the anti-MAG antibody response in two patients with anti-MAG neuropathy that were treated with rituximab for several years. A reduction of 50% in the anti-MAG IgM was seen after 19 and 58 months respectively, and of 70% after 74 or 104 months of treatment respectively. Titres remained low, without evidence of recurrence after the treatments were discontinued.
UNASSIGNED: Therapy of anti-MAG neuropathy with rituximab may require repeat treatments over more than one year to achieve a significant reduction in autoantibody concentrations. These considerations should inform treatment decisions and the design of clinical trials.
摘要：
抗MAG神经病是一种缓慢进行性脱髓鞘神经病，可导致残疾。神经病被认为是由靶向周围神经中的髓磷脂相关糖蛋白(MAG)的单克隆IgM抗体引起的。治疗旨在降低B细胞消耗剂的自身抗体浓度，最常见的是利妥昔单抗，基于病例系列和报告改善的非受控试验。没有FDA批准的抗MAG神经病的治疗方法，然而,两项持续时间相对较短的利妥昔单抗随机对照试验未能达到预定的主要终点.关于有效降低抗体浓度所需的治疗次数或持续时间的信息也很少。
■我们报告了使用利妥昔单抗治疗数年的两名抗MAG神经病变患者的抗MAG抗体反应的时程。在19和58个月后，抗MAGIgM分别减少了50％，在74或104个月的治疗后分别为70％。滴度仍然很低，治疗停止后无复发证据.
用利妥昔单抗治疗抗MAG神经病可能需要在一年以上的时间内重复治疗以实现自身抗体浓度的显着降低。这些考虑因素应告知治疗决策和临床试验的设计。
■我们报告了使用利妥昔单抗治疗数年的两名抗MAG神经病变患者的抗MAG抗体反应的时程。在19和58个月后，抗MAGIgM分别减少了50％，在74或104个月的治疗后分别为70％。滴度仍然很低，治疗停止后无复发证据.
用利妥昔单抗治疗抗MAG神经病可能需要在一年以上的时间内重复治疗以实现自身抗体浓度的显着降低。这些考虑因素应告知治疗决策和临床试验的设计。
