全血灌流免疫吸附治疗被动转移型实验性自身免疫性重症肌无力的研究

Treatment of passively transferred experimental autoimmune myasthenia gravis by extracorporeal whole blood immunoadsorption

目的观察全血灌流免疫吸附治疗实验性自身免疫性重症肌无力(EAMG)的疗效。方法用重症肌无力患者血清中的免疫球蛋白(IgG)建立急性EAMG模型,并进行全血灌流免疫吸附治疗,观察其疗效。结果以球形纤维素为载体色氨酸为配基的吸附剂治疗2h,能清除49.85%±2.55%的致病抗体,肌无力症状显著改善;重复神经电刺激实验CMAP衰减率从21.875±3.226(3Hz)、22.250±2.815(5Hz)和24.375±1.685(10Hz)恢复到17.875±1.642(P<0.05)、18.750±1.388(P<0.05)和23.250±1.388;单纤维肌电图示MCD值也从23.125±2.997(3Hz)、26.375±4.172(5Hz)和30.375±4.470(10Hz)显著性地缩短至19.250±1.488(P<0.05)、21.750±2.375(P<0.05)和26.125±2.031(P<0.05)MEPP波幅(μV)也从30.54±3.74显著增加至40.43±3.45(P<0.01),频率(次/s)从17.18±2.87显著增加至18.74±2.75(P<0.01);单位面积的神经肌肉接头数从9.825±3.401增加至10.900±2.879(P<0.05)。结论全血灌流免疫吸附治疗能有效清除被动转移型重症肌无力动物体内致病抗体,并相应改善临床症状,提高神经肌肉电传导功能,增加神经肌肉接头处nAChR数量,为临床重症肌无力的血液净化治疗提供了新措施。

Objective To investigate the effects of extracorporeal whole blood immunoadsorption in the treatment of passively transferred experimental autoimmune myasthenia gravis. Methods EAMG rabbits were induced by passively transferred the anti-acetylcholine receptor antibody of myasthenia gravis patients and treated with extracorporeal whole blood adsorption. The improvement of clinical manifestation was evaluated through clinical score,antibody titers,neuromuscular transmission function and neuromuscular junction quantity. Results The pathogenic antibodies were removed by 49. 85%±2. 55% at 2h after treatment. The clinical manifestation improved 3 days after the immunoadsorption. Repetitive nerve stimulation test showed that the percentage of decrement of CMAP dropped from 21. 875±3. 226（3Hz）,22. 250±2. 815（5Hz） and 24. 375±1. 685（10Hz） to 17. 875±1. 642 （P 〈 0.05 ）, 18. 750 ± 1. 388 （P 〈 0.05） and 23. 250 ± 1. 388, respectively. Single fiber electromyography showed that MCD（μs） decreased significantly from 23. 125 ± 2. 997 （3Hz）, 26. 375 ± 4. 172 （5Hz） and 30. 375 ± 4.470（10Hz） to 19.250±1.488 （P〈0.05）,21.750±2.375 （P〈0.05） and 26.125±2.031（P〈0.05）. The amplitude （μV） of miniature endplate potential（MEPP） increased significantly from 30.54±3.74 to 40.43±3.45 （P〈0. 01）. The frequency of MEPP also increased significantly from 17.18±2.87 to 18.74±2.75（P〈0. 01）. The quantity of neuromuscular junction per unit area （25mm^2） increased from 9. 825±3. 401 to 10. 900±2. 879（P〈 0.05）. Conclusion Extracorporeal whole blood immunoadsorption is very effective in treating the passive experimental autoimmune myasthenia gravis by improving clinical manifestation ,antibody titers ,neuromuscular transmission function and neuromuscular junction quantity.