Soueidan S A, Dalakas M C
Neuromuscular Diseases Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892.
Pediatr Res. 1993 Jan;33(1 Suppl):S95-100. doi: 10.1203/00006450-199333011-00017.
The majority of autoimmune neuromuscular diseases fall into three groups: 1) The autoimmune neuropathies, which include the acute inflammatory demyelinating polyneuropathy (Guillain-Barré syndrome), the chronic inflammatory demyelinating polyneuropathy, the paraproteinemic polyneuropathies, and the anti-GM1-associated motor neuropathies with conduction block; 2) the inflammatory myopathies, which include the dermatomyositis and polymyositis complex; and 3) the autoimmune neuromuscular junction defects, which include myasthenia gravis, and the Lambert-Eaton myasthenic syndrome. Laboratory and clinical evidence suggests that circulating antibodies or sensitized lymphocytes are operating in the pathogenesis of these conditions. Current immunotherapies include treatment with plasmapheresis, high-dose steroids, or immunosuppressive drugs. Although all of these therapies are effective in a number of patients and for some period of time, they often result in serious side effects that necessitate their discontinuation. The need for safer and more effective therapies in the treatment of these conditions prompted the use of high-dose i.v. immune globulin (IVIG). A number of small trials and a few reports suggest that IVIG is safe and effective in the treatment of patients with autoimmune neuropathies, inflammatory myopathies, and myasthenia gravis unresponsive to conventional therapies. We will present current experience with IVIG in the above-mentioned autoimmune neuromuscular diseases, and we will stress the need for long-term controlled studies. The possible immunomodulatory action of IVIG in these conditions will also be discussed.
1）自身免疫性神经病，包括急性炎症性脱髓鞘性多发性神经病（吉兰-巴雷综合征）、慢性炎症性脱髓鞘性多发性神经病、副蛋白血症性多发性神经病以及伴有传导阻滞的抗GM1相关运动神经病；2）炎症性肌病，包括皮肌炎和多发性肌炎综合征；3）自身免疫性神经肌肉接头缺陷，包括重症肌无力和兰伯特-伊顿肌无力综合征。实验室和临床证据表明，循环抗体或致敏淋巴细胞在这些疾病的发病机制中起作用。目前的免疫疗法包括血浆置换、大剂量类固醇或免疫抑制药物治疗。尽管所有这些疗法在许多患者中在一段时间内都是有效的，但它们常常导致严重的副作用，从而需要停药。在治疗这些疾病时，需要更安全、更有效的疗法，这促使人们使用大剂量静脉注射免疫球蛋白（IVIG）。一些小型试验和一些报告表明，IVIG在治疗对传统疗法无反应的自身免疫性神经病、炎症性肌病和重症肌无力患者中是安全有效的。我们将介绍目前在上述自身免疫性神经肌肉疾病中使用IVIG的经验，并强调长期对照研究的必要性。还将讨论IVIG在这些疾病中可能的免疫调节作用。
