Dalakas M C
Neuromuscular Disease Section, NINDS, National Institutes of Health, Bethesda, Maryland 20892.
Semin Neurol. 1994 Jun;14(2):137-45. doi: 10.1055/s-2008-1041071.
The inflammatory myopathies include 3 distinct entities, PM, DM, and IBM. These diseases differ clinically, immunopathologically, and in their response to therapies. Although DM and IBM are easy to diagnose on the basis of characteristic clinicopathologic findings, PM still remains a diagnosis of exclusion. A T cell-mediated cytotoxic process in PM and IBM and a complement-mediated microangiopathy in DM, along with the various serologic markers of autoimmunity, are the hallmarks of the underlying autoimmune processes in these groups. Although in uncontrolled studies PM and DM appear to respond to prednisone and immunosuppressive drugs to some degree and for some period of time, IBM is resistant to all therapies. Currently, high-dose intravenous immunoglobulin (IVIG) appears to be an encouraging and safe new modality of treatment for some of these conditions when other therapies have failed. In a controlled study, IVIG has been shown to be effective in DM and, in uncontrolled studies, in some patients with PM or IBM.
炎性肌病包括三种不同的类型，即多发性肌炎（PM）、皮肌炎（DM）和包涵体肌炎（IBM）。这些疾病在临床、免疫病理学及对治疗的反应方面存在差异。虽然DM和IBM基于特征性的临床病理表现易于诊断，但PM仍然是一种排除性诊断。PM和IBM中以T细胞介导的细胞毒性过程以及DM中补体介导的微血管病，连同各种自身免疫血清学标志物，是这些疾病潜在自身免疫过程的标志。尽管在非对照研究中，PM和DM在一定程度上且在一段时间内似乎对泼尼松和免疫抑制药物有反应，但IBM对所有治疗均耐药。目前，当其他治疗方法失败时，大剂量静脉注射免疫球蛋白（IVIG）似乎是治疗其中一些疾病的一种令人鼓舞且安全的新方法。在一项对照研究中，IVIG已被证明对DM有效，在非对照研究中，对一些PM或IBM患者也有效。
