Hughes Richard
Cochrane Neuromuscular Disease Review Group, Department of Clinical Neuroscience, King's College London, Guy's Hospital, London, SE1 1UL, UK.
J Neurol. 2008 Jul;255 Suppl 3:7-11. doi: 10.1007/s00415-008-3003-z.
Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN) are the major immune neuropathies. Although a detailed understanding of the pathogenesis of these conditions is not yet available, the multiple effects of IVIg on the immune and inflammatory process recommend it as an agent worthy of investigation in these diseases. Following recent research, IVIg is now recommended as a first-line treatment option for moderate or severe GBS to be administered within two weeks of disease onset. With regard to CIDP, a Cochrane review demonstrated significant short-term improvements in disability and impairment with IVIg. The ICE (IGIV CIDP Efficacy trial) study group undertook the largest ever trial of IVIg for CIDP, which demonstrated for the first time the long-term efficacy of IVIg. The results of this ICE trial demonstrated the efficacy of IVIg in CIDP, with a significantly higher response rate versus placebo after 24 weeks of treatment (P = 0.0002). Furthermore, long-term maintenance with IVIg also significantly reduced the rate of relapse (P < 0.011). On the basis of available data, IVIg can be recommended as a first-line treatment option for GBS and CIDP. For MMN, although the evidence for IVIg is limited, there is no evidence to recommend other treatments.
吉兰-巴雷综合征(GBS)、慢性炎症性脱髓鞘性多发性神经根神经病(CIDP)和多灶性运动神经病(MMN)是主要的免疫性神经病。尽管目前对这些疾病的发病机制尚无详细了解,但静脉注射免疫球蛋白(IVIg)对免疫和炎症过程的多种作用使其成为值得在这些疾病中进行研究的药物。根据最近的研究,IVIg现在被推荐作为中度或重度GBS的一线治疗选择,应在疾病发作后两周内给药。关于CIDP,一项Cochrane综述表明,IVIg可使残疾和功能障碍在短期内得到显著改善。ICE(静脉注射免疫球蛋白治疗CIDP疗效试验)研究小组进行了有史以来最大规模的IVIg治疗CIDP试验,首次证明了IVIg的长期疗效。ICE试验结果表明,IVIg对CIDP有效,治疗24周后,其有效率显著高于安慰剂(P = 0.0002)。此外,长期使用IVIg维持治疗也显著降低了复发率(P < 0.011)。根据现有数据,IVIg可被推荐作为GBS和CIDP的一线治疗选择。对于MMN,尽管IVIg的证据有限,但没有证据推荐其他治疗方法。