Therapeutic approaches in patients with inflammatory myopathies.
Therapeutic approaches in patients with inflammatory myopathies.
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炎症性肌病患者的治疗方法。
DOI：
10.1055/s-2003-41136
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发表时间：
2003
影响因子：
2.7
通讯作者：
Dalakas,MarinosC
中科院分区：
文献类型：
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作者：
Dalakas,MarinosC
在炎症性肌病中，皮肌炎(DM)仍然是最可治疗的亚型，在大多数情况下，对类固醇、静脉注射免疫球蛋白(IVIg)或免疫抑制剂有反应。包涵体肌炎(IBM)仍然是最难治疗的疾病；在非对照研究中，免疫抑制剂和类固醇没有帮助，静脉注射免疫球蛋白的对照试验也令人失望。多发性肌炎(PM)是一种非常罕见的疾病，尽管仍被过度诊断，但它的罕见给进行大规模治疗研究带来了困难；然而，基于小系列，PM似乎对免疫治疗干预有不同的反应。炎症性肌病治疗中最一致的问题仍然是真正的PM与难以治疗的IBM病例或与继发性肌内膜炎症可能突出的坏死性肌病和营养不良过程的区别。由于针对T细胞激活分子、细胞因子、趋化因子和黏附受体的新药物的可获得性，PM、DM和IBM的未来管理似乎是有希望的。在IBM，这种免疫调节药物的使用可以与阻断细胞因子增强的淀粉样蛋白的药物或抑制淀粉样蛋白纤维的形成和聚合的药物相结合。
Among the group of inflammatory myopathies, dermatomyositis (DM) remains the most treatable subset responding, in the majority of the cases, to steroids, intravenous immunoglobulin (IVIg), or immunosuppressants. Inclusion-body myositis (IBM) remains the most difficult disease to treat; in uncontrolled studies immunosuppressants and steroids have not helped, and controlled trials with IVIg have been disappointing. Polymyositis (PM) is a very uncommon, although still overdiagnosed, disorder and its rarity poses difficulties in performing large-scale therapeutic studies; based on small series, however, PM seems to variably respond to immunotherapeutic interventions. The most consistent problem in the treatment of inflammatory myopathies remains the distinction of true PM from the difficult-to-treat cases of IBM, or from necrotizing myopathies and dystrophic processes where secondary endomysial inflammation may be prominent. The future in the management of PM, DM, and IBM seems promising because of the availability of new agents directed at T-cell activation molecules, cytokines, chemokines, and adhesion receptors. In IBM, the use of such immunomodulatory drugs may be combined with agents that block cytokine-enhancing amyloid or with agents that inhibit the formation and polymerization of amyloid fibrils.