血浆置换治疗进展型多发性硬化症和nmo谱系疾病的临床疗效

P. Petrou, T. Ben-Hur, A. Vaknin-Dembinsky, O. Abramsky, D. Karussis
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引用次数: 3

摘要

背景:血浆置换/血浆置换(PLEX)是治疗多种免疫介导性疾病的有效方法。除了已知的对重症肌无力和Gulliain Barre综合征的疗效外,它也被证明对某些多发性硬化症和其他中枢神经系统脱髓鞘疾病患者在疾病急性/亚急性恶化期间有效。目的和方法:我们报告了一项开放性前瞻性研究的结果,该研究对36例进行性多发性硬化症(继发性进行性或复发进行性)和12例nmo谱系疾病患者进行了PLEX治疗。所有患者在纳入研究前一年都经历了明显的临床恶化(EDSS量表中0.5-1度或以上,或严重复发,但没有完全恢复),并且对类固醇治疗部分或根本没有反应。纳入时平均EDSS评分为5.91±1.46。MS亚组平均EDSS为5.95±1.3,NMO亚组平均EDSS为5.6±1.4。平均病程11.4±7.8年(MS为12±7.6年,NMO为5.75±4.59年)。所有患者均在2周内接受5个疗程的PLEX治疗,随后每月一次,持续1年。结果:48例患者中有28例(58.3%)在开始使用PLEX后的一年内EDSS评分显著改善。平均EDSS评分从入组时的5.91±1.46下降到一年后的5.41±1.8。这种改善在NMO组更为明显:12例NMO患者中有10例(83%)改善,其平均EDSS评分从治疗前的5.6±1.4降至PLEX后的4.7±1.5。在整个组中,有16例患者过度复发(复发-进展过程),在纳入前一年共26例复发;在PLEX之后的一年里,复发的次数从26次减少到4次。一般来说,有明显髓鞘受累的患者对治疗有最令人印象深刻的反应。5例患者出现轻微感染,1例因败血症入院。没有观察到其他主要副作用。结论:PLEX可能使一些进展性MS和NMO患者受益,因此对于这些疾病高度活跃的患者,特别是那些髓样形式的患者,以及近期恶化而类固醇无反应的患者,可能是一种替代的二线治疗方式。有必要进行更大规模的对照研究,以证实PLEX在这些MS亚组中的疗效。
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Clinical Efficacy of Plasma-Exchange in Patients with Progressive forms of Multiple Sclerosis and NMO-Spectrum Disease
Background: Plasma-exchange/plasmapheresis (PLEX) is an efficient treatment for several immune mediated diseases. In addition to its known efficacy in myasthenia gravis and Gulliain Barre syndrome, it has been also shown to be effective in certain patients with MS and other CNS demyelinating disorders, during an acute/sub-acute deterioration of the disease. Aims and methods: We report the results of an open prospective study with PLEX in 36 patients with progressive forms of multiple sclerosis (either secondary progressive or relapsing-progressive) and 12 patients with NMO-spectrum disease. All patients had experienced a significant clinical deterioration in the year prior to inclusion (0.5-1 degree or more, in the EDSS scale or a severe relapse from which they did not fully recover) and responded partially or not at all to steroidal treatment. The mean EDSS score at inclusion was 5.91 ± 1.46. The mean EDSS for the MS subgroup was 5.95 ± 1.3 and for the NMO subgroup, 5.6 ± 1.4. The mean duration of the disease was 11.4 ± 7.8 years (12 ± 7.6 for the MS and 5.75 4.59 for the NMO). All patients were treated with 5 courses of PLEX in 2 weeks, followed by a monthly course for one year. Results: Twenty eight of the 48 patients (58.3 %) improved significantly in the EDSS score at year one post initiation of PLEX. The mean EDSS score declined from 5.91 ± 1.46 at inclusion, to 5.41 ± 1.8 at year one. This improvement was more pronounced in the NMO group: Ten out of twelve patients with NMO (83%) improved and their mean EDSS score was reduced from 5.6 ± 1.4 before the treatment to 4.7 ± 1.5 EDSS score post PLEX. In the whole group there were 16 patients with over imposed relapses (relapsing-progressive course) with a total of 26 relapses in the year prior to the inclusion; the number of relapses during the year following PLEX was reduced from 26 to 4. In general patients with prominent myelitic involvement had the most impressive response to the treatment. Five patients suffered from minor infections and one was admitted with sepsis. No other major side effects were observed. Conclusion: PLEX may benefit some patients with progressive MS and NMO and thus may represent an alternative second line treatment modality for such patients with highly active disease, especially those with myelitic forms, and recent deterioration that did not respond to steroids. Larger, controlled studies are warranted to confirm the efficacy of PLEX in these subgroups of MS.
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