Department of Neurosciences, Imperial College, London, UK.
Curr Opin Pharmacol. 2010 Jun;10(3):346-52. doi: 10.1016/j.coph.2010.03.001. Epub 2010 Apr 19.
Based on unique clinicopathological criteria, the most common immune inflammatory muscle disorders include Dermatomyositis (DM), Polymyositis (PM), Necrotizing Myositis (NM), and sporadic Inclusion Body Myositis (sIBM). DM is an undeniably a complement-mediated microangiopathy with destruction of capillaries, hypoperfusion, and inflammatory cell stress on the perifascicular regions. Necrotizing Myopathy is a poorly studied subacute myopathy triggered by toxic, viral, or autoimmune factors with macrophages as the final effector cells. In PM and IBM cytotoxic CD8-positive T-cells clonally expand in situ and invade MHC-I-expressing muscle fibers. In sIBM, in addition to autoimmune inflammation, there are degenerative features characterized by vacuolization and accumulation of stressor and amyloid-related molecules. Advances in the immunobiology of these disorders are discussed including the interaction between pro-inflammatory and beta-amyloid or stressor proteins. A critical review regarding tissue biomarkers and strategies for more effective treatments are presented.
基于独特的临床病理标准，最常见的免疫炎症性肌肉疾病包括皮肌炎（DM）、多发性肌炎（PM）、坏死性肌病（NM）和散发性包涵体肌炎（sIBM）。DM 是一种不可否认的补体介导的微血管病，伴有毛细血管破坏、灌注不足和血管周围区域的炎症细胞应激。坏死性肌病是一种研究甚少的亚急性肌病，由毒性、病毒或自身免疫因素触发，巨噬细胞是最终的效应细胞。在 PM 和 IBM 中，细胞毒性 CD8 阳性 T 细胞原位克隆扩增并侵入 MHC-I 表达的肌纤维。在 sIBM 中，除了自身免疫炎症外，还存在退行性特征，表现为空泡化和应激和淀粉样相关分子的积累。本文讨论了这些疾病的免疫生物学进展，包括促炎和β-淀粉样或应激蛋白之间的相互作用。还提出了关于组织生物标志物和更有效治疗策略的批判性综述。
