Intravenous immune globulin therapy for neurologic diseases
Intravenous immune globulin therapy for neurologic diseases
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DOI：
10.7326/0003-4819-126-9-199705010-00008
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发表时间：
1997-05-01
影响因子：
39.2
通讯作者：
Dalakas, MC
中科院分区：
文献类型：
--
作者：
Dalakas, MC
大剂量静脉注射免疫球蛋白(IVIg)已成为治疗各种神经系统疾病的重要手段。对临床试验结果的不同解释；IVIg与替代疗法相比的预期好处；以及关于IVIg的安全性、成本和作用机制的问题，引起了从业者的担忧和不确定性。为了阐明这些方面，本文研究了过去6年在美国国家神经疾病和中风研究所接受IVIg治疗的110多名各种自身免疫性神经疾病患者的临床、血清学和免疫学数据。在对照临床试验中，IVIg已有效地治疗格林-巴利综合征、多灶性运动神经病、慢性炎症性脱髓鞘多神经病和皮肌炎。在其他对照或开放标签试验和病例报告中，IVIg对几名Lambert-Eaton肌无力综合征和重症肌无力患者产生了改善，但对一些包涵体肌炎、副蛋白血症IgM脱髓鞘多神经病、某些顽固性儿童癫痫、多发性肌炎、多发性硬化症、视神经炎和僵尸综合征患者有可变的、轻微的或未经证实的益处。主要的不良反应是头痛；很少发生无菌性脑膜炎、皮肤反应、血栓栓子事件和肾小管坏死。IVIg最相关的免疫调节作用，无论是单独作用还是联合作用，都是抑制补体沉积，中和细胞因子，调节Fc受体介导的吞噬作用，以及下调自身抗体的产生。静脉注射免疫球蛋白治疗某些自身免疫性神经系统疾病是有效的，但其疗效范围尚未完全确定。还需要进行更多的临床对照试验。
High-dose intravenous immune globulin (IVIg) has emerged as an important therapy for various neurologic diseases. Different interpretations of clinical trial results; the expected benefit of IVIg compared with that of alternate therapies; and issues about IVIg's safety, cost, and mechanisms of action have raised concern and uncertainty among practitioners. To clarify these areas, this paper examines the clinical, serologic, and immunologic data on more than 110 patients with various autoimmune neurologic diseases who received IVIg during the past 6 years at the National Institute of Neurological Disorders and Stroke. It also reviews work by other investigators on the efficacy, risks, benefits, and mechanisms of the action of IVIg in these diseases.In controlled clinical trials, IVIg has been effective in treating the Guillain-Barre syndrome, multifocal motor neuropathy, chronic inflammatory demyelinating polyneuropathy, and dermatomyositis. In other controlled or open-label trials and case reports, IVIg produced improvement in several patients with the Lambert-Eaton myasthenic syndrome and myasthenia gravis but had a variable, mild, or unsubstantiated benefit in some patients with inclusion-body myositis, paraproteinemic IgM demyelinating polyneuropathy, certain intractable childhood epilepsies, polymyositis, multiple sclerosis, optic neuritis, and the stiff-man syndrome. The primary adverse reaction was headache; aseptic meningitis, skin reactions, thromboembolic events, and renal tubular necrosis occurred rarely. The most relevant immunomodulatory actions of IVIg, operating alone or in combination, are inhibition of complement deposition, neutralization of cytokines, modulation of Fc-receptor-mediated phagocytosis, and downregulation of autoantibody production. Therapy with IVIg is effective for certain autoimmune neurologic diseases, but its spectrum of efficacy has not been fully established. Additional controlled clinical trials are needed.