Dalakas Marinos C
Neuromuscular Diseases Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Building 10, Room 4N248, 10 Center Drive, MSC 1382, Bethesda, MD 20892-1382, USA.
Semin Neurol. 2003 Jun;23(2):199-206. doi: 10.1055/s-2003-41136.
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.
在炎性肌病组中，皮肌炎（DM）仍是最可治疗的亚型，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，IVIg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，尽管仍存在过度诊断的情况，其罕见性给进行大规模治疗研究带来了困难；然而，基于小样本系列研究，PM似乎对免疫治疗干预有不同程度的反应。炎性肌病治疗中最一致的问题仍然是如何将真正的PM与难以治疗的IBM病例、坏死性肌病以及肌内膜继发性炎症可能突出的营养不良性病变区分开来。由于有针对T细胞激活分子、细胞因子、趋化因子和黏附受体的新型药物，PM、DM和IBM的治疗前景似乎很乐观。在IBM中，使用此类免疫调节药物可与阻断细胞因子增强淀粉样蛋白的药物或抑制淀粉样蛋白原纤维形成和聚合的药物联合使用。
