van der Meché F G, van Doorn P A
Department of Neurology, University Hospital and Erasmus University, Rotterdam, The Netherlands.
Muscle Nerve. 1997 Feb;20(2):136-47. doi: 10.1002/(sici)1097-4598(199702)20:2<136::aid-mus1>3.0.co;2-d.
High-dose immunoglobulins for intravenous administration (IVIg) have originally been developed for substitution therapy in hypogammaglobulinemia. Over the last decade they are increasingly used in the treatment of immune-mediated diseases. In this review the results in immune-mediated neuromuscular diseases are summarized. Positive effects are demonstrated in open studies in dermato- and polymyositis, myasthenia gravis, and inflammatory neuropathies. Properly conducted randomized clinical trials demonstrating the effect of IVIg are available in dermatomyositis, Guillain-Barré syndrome, and chronic inflammatory demyelinating polyneuropathy, and smaller ones in multifocal motor neuropathy. In myasthenia gravis a trial is at present underway and only interim results are available. The results of a trial in the Lambert-Eaton myasthenic syndrome are in the process of publication. The therapeutic approach in individual patients is discussed, but often appears to be difficult. Considering chronic treatment with IVIg, proper long-term studies including cost-benefit studies are needed. Future developments aim for combination therapies, since IVIg and immune suppressants like prednisone are suggested to have a synergistic effect.
静脉注射用高剂量免疫球蛋白（IVIg）最初是为低丙种球蛋白血症的替代疗法而开发的。在过去十年中，它们越来越多地用于免疫介导疾病的治疗。在这篇综述中，总结了免疫介导的神经肌肉疾病的治疗结果。在皮肌炎和多发性肌炎、重症肌无力和炎性神经病的开放性研究中显示出积极效果。在皮肌炎、格林-巴利综合征和慢性炎性脱髓鞘性多发性神经病中开展了能证明IVIg疗效的恰当随机临床试验，在多灶性运动神经病中也开展了规模较小的试验。在重症肌无力方面，目前正在进行一项试验，仅有中期结果。兰伯特-伊顿肌无力综合征的一项试验结果正在发表过程中。文中讨论了个体患者的治疗方法，但通常似乎很困难。考虑到IVIg的长期治疗，需要进行包括成本效益研究在内的恰当长期研究。未来的发展目标是联合治疗，因为IVIg和泼尼松等免疫抑制剂被认为具有协同作用。
