van Schaik Ivo N
Department of Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
J Peripher Nerv Syst. 2008 Dec;13(4):258-60. doi: 10.1111/j.1529-8027.2008.00189.x.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)-related research has made progress in the field of pathogenesis, genetics, and treatment. The number of circulating CD4(+) CD25(+) T-regulatory cells was shown to be reduced in CIDP patients. Increased frequency of genotype GA13-16 of the SH2D2A gene encoding for a T-cell-specific adapter protein in CIDP patients may result in a defective control and elimination of autoreactive T cells. IVIg treatment has been shown to increase numbers and function of peripheral CD4(+) CD25(+) T-regulatory cell in a mouse model. These findings shed new light on the understanding of why peripheral tolerance is breached in CIDP patients and why the disease becomes chronic and adds another possible mechanism of action of intravenous immunoglobulin to the already long list. Long-term effectiveness of IVIg has now been proven. Subcutaneous immunoglobulin could be an alternative for IVIg, but this has to be explored further in well-designed trials. Autologous stem cell transplantation has been tried in refractory patients, but larger trials are necessary to assess safety and effect of this treatment.
慢性炎症性脱髓鞘性多发性神经根神经病（CIDP）相关研究在发病机制、遗传学和治疗领域取得了进展。CIDP患者循环中CD4(+) CD25(+) T调节细胞数量减少。CIDP患者中编码T细胞特异性衔接蛋白的SH2D2A基因的GA13 - 16基因型频率增加，可能导致对自身反应性T细胞的控制和清除存在缺陷。在小鼠模型中，静脉注射免疫球蛋白（IVIg）治疗已显示可增加外周CD4(+) CD25(+) T调节细胞的数量和功能。这些发现为理解CIDP患者为何外周耐受性被破坏以及疾病为何变为慢性提供了新线索，并在已有的众多作用机制中又增加了静脉注射免疫球蛋白的一种可能作用机制。现已证实IVIg的长期有效性。皮下注射免疫球蛋白可能是IVIg的替代方法，但这必须在精心设计的试验中进一步探索。自体干细胞移植已在难治性患者中尝试，但需要更大规模的试验来评估这种治疗的安全性和效果。
