Department of Neurology and Rehabilitation, University of Illinois Medical Center, 912 South Wood Street, 855N, M/C 796, Chicago, Illinois 60612, USA.
Muscle Nerve. 2012 Sep;46(3):449-53. doi: 10.1002/mus.23488.
In this study we describe a patient with a prolonged myasthenic crisis refractory to conventional immunomodulatory therapy who was treated with GM-CSF (granulocyte macrophage colony-stimulating factor, sargramostim).
T-regulatory cell (Treg) suppressive function and Foxp3 expression were evaluated before and after treatment with GM-CSF.
Treatment with GM-CSF was associated with clinical improvement, expansion in the circulating numbers of Foxp3(+) cells, increase in Foxp3 expression levels in Tregs, early improvement in Treg suppressive capacity for AChR-α-induced T-cell proliferation, and subsequent enhancement in Treg suppression of polyclonal T-cell proliferation.
Although definitive conclusions cannot be drawn from a single case, the correlation with similar findings in GM-CSF-treated animals with experimental autoimmune myasthenia gravis suggests further exploration of the effects of GM-CSF in myasthenia gravis should be studied in a clinical trial setting.
在本研究中,我们描述了一例重症肌无力危象患者,该患者对常规免疫调节治疗无反应,接受了 GM-CSF(粒细胞巨噬细胞集落刺激因子,沙格司亭)治疗。
在接受 GM-CSF 治疗前后,评估了调节性 T 细胞(Treg)的抑制功能和 Foxp3 表达。
GM-CSF 治疗与临床改善相关,循环中 Foxp3(+)细胞数量增加,Treg 中 Foxp3 表达水平升高,AChR-α诱导的 T 细胞增殖的 Treg 抑制作用早期改善,随后增强了 Treg 对多克隆 T 细胞增殖的抑制作用。
尽管不能从单个病例得出明确结论,但与实验性自身免疫性重症肌无力中 GM-CSF 治疗动物的类似发现相关,提示应在临床试验中进一步研究 GM-CSF 在重症肌无力中的作用。