Mastaglia F L, Phillips B A, Zilko P
Australian Neuromuscular Research Institute and Department of Medicine, Queen Elizabeth II Medical Centre, Perth, Australia.
Muscle Nerve. 1997 Jun;20(6):651-64. doi: 10.1002/(sici)1097-4598(199706)20:6<651::aid-mus1>3.0.co;2-7.
The treatment of the immune-mediated inflammatory myopathies remains largely empirical. Corticosteroids are usually effective in polymyositis and dermatomyositis but may need to be combined with methotrexate or azathioprine in some patients. Intravenous immunoglobulin (IVIg) is effective as add-on therapy in some patients not adequately controlled with steroids or immunosuppressive agents, but further controlled trials of IVIg are necessary to define the indications and optimal dose regimens. Cyclophosphamide, cyclosporin, or chlorambucil may be effective in patients with refractory polymyositis or dermatomyositis. Low-dose whole body or lymphoid irradiation is a last option in severely disabled patients resistant to all other treatments. As a small proportion of patients with inclusion body myositis respond to corticosteroid or immunosuppressive therapy, a 3-6-month trial of such therapy is justified in this condition. More specific immunotherapy for these disorders awaits identification of the target antigens and further clarification of the immunopathogenetic mechanisms.
免疫介导性炎性肌病的治疗很大程度上仍基于经验。皮质类固醇通常对多发性肌炎和皮肌炎有效，但在某些患者中可能需要与甲氨蝶呤或硫唑嘌呤联合使用。静脉注射免疫球蛋白（IVIg）作为附加疗法，对一些未用类固醇或免疫抑制剂充分控制的患者有效，但需要进一步的对照试验来确定其适应症和最佳剂量方案。环磷酰胺、环孢素或苯丁酸氮芥可能对难治性多发性肌炎或皮肌炎患者有效。低剂量全身或淋巴照射是对所有其他治疗均耐药的严重残疾患者的最后选择。由于一小部分包涵体肌炎患者对皮质类固醇或免疫抑制疗法有反应，因此在这种情况下进行3至6个月的此类疗法试验是合理的。针对这些疾病的更特异性免疫疗法有待确定靶抗原并进一步阐明免疫发病机制。
