Dalakas M C
Neuromuscular Diseases Section, National Institute of Neurological Disorders and Stroke, Bethesda, MD 20892-1382, USA.
Clin Exp Immunol. 1996 May;104 Suppl 1:55-60.
High-dose intravenous immune globulin (IVIG) is emerging as a promising therapy for patients with inflammatory myopathies who have become unresponsive to, or cannot tolerate, conventional therapies. In a double-blind, placebo-controlled study, using objective criteria for improvement, IVIG demonstrated moderate to dramatic improvement in 75% of the patients with dermatomyositis. Preliminary results from a controlled study in inclusion-body myositis show that IVIG may also exert a mild benefit, but only in a small number of patients and in certain muscle groups. In some patients with polymyositis, IVIG is reported to be of benefit but controlled studies have not yet been completed. Immunocytochemical, immunological and in vitro studies on the patients' repeated muscle biopsies and follow-up sera showed that IVIG exerts its action in inflammatory myopathies by: (i) inhibiting myotoxic cytokines, such as TNF-alpha and IL-1; (ii) blockade of Fc receptors on endomysial macrophages interfering with Fc receptor-mediated phagocytosis; and (iii) inhibiting the uptake of C3 and intercepting the formation and deposition of membranolytic attack complex on the endomysial capillaries.
大剂量静脉注射免疫球蛋白（IVIG）正在成为一种有前景的治疗方法，用于那些对传统疗法无反应或无法耐受的炎性肌病患者。在一项双盲、安慰剂对照研究中，根据改善的客观标准，IVIG在75%的皮肌炎患者中显示出中度到显著的改善。一项关于包涵体肌炎的对照研究的初步结果表明，IVIG可能也有轻微益处，但仅在少数患者和某些肌肉群中如此。据报道，IVIG对一些多发性肌炎患者有益，但对照研究尚未完成。对患者重复进行的肌肉活检和随访血清进行的免疫细胞化学、免疫学和体外研究表明，IVIG通过以下方式在炎性肌病中发挥作用：（i）抑制肌毒性细胞因子，如肿瘤坏死因子-α和白细胞介素-1；（ii）阻断肌内膜巨噬细胞上的Fc受体，干扰Fc受体介导的吞噬作用；（iii）抑制C3的摄取并阻断膜溶解性攻击复合物在肌内膜毛细血管上的形成和沉积。
