Department of Neurology, Pusan National University School of Medicine, Yangsan, Korea.
J Korean Med Sci. 2009 Dec;24(6):1015-23. doi: 10.3346/jkms.2009.24.6.1015. Epub 2009 Nov 7.
This study was performed in order to characterize the types of the infiltrating cells, and the expression profiles of major histocompatibility complex (MHC) class I and membrane attack complex (MAC) in patients with inflammatory myopathies and dysferlinopathy. Immunohistochemical stains were performed using monoclonal antibodies against several inflammatory cell types, MHC class I, and MAC in muscles from inflammatory myopathies and dysferlinopathy. There was significant difference in the types of infiltrating cells between polymyositis (PM), dermatomyositis (DM), and dysferlinopathy, including significantly high CD4+/CD8+ T cell ratio and B/T cell ratio in DM. In dysferlinopathy, CD4+ T cells were the most abundant and the proportions of infiltrating cell types were similar to those of DM. MHC class I was expressed in muscle fibers of PM and DM regardless of the presence of inflammatory infiltrates. MAC was expressed in necrotic fibers and vessels of PM and DM. One patient with early stage DM had a MAC deposits on endomysial capillaries. In dysferlinopathy, MAC deposit was also observed on the sarcolemma of nonnecrotic fibers. The analysis of inflammatory cells, MHC class I expressions and MAC deposits may help to differentiate dysferlinopathy from idiopathic inflammatory myopathy.
本研究旨在描述炎症性肌病和肌营养不良症患者浸润细胞的类型，以及主要组织相容性复合体（MHC）I 类和膜攻击复合物（MAC）的表达谱。使用针对几种炎症细胞类型、MHC I 类和 MAC 的单克隆抗体对炎症性肌病和肌营养不良症的肌肉进行免疫组织化学染色。多发性肌炎（PM）、皮肌炎（DM）和肌营养不良症之间浸润细胞的类型存在显著差异，DM 中 CD4+/CD8+T 细胞比值和 B/T 细胞比值明显升高。在肌营养不良症中，CD4+T 细胞最为丰富，浸润细胞类型的比例与 DM 相似。MHC I 类在 PM 和 DM 的肌纤维中表达，无论是否存在炎症浸润。MAC 在 PM 和 DM 的坏死纤维和血管中表达。1 例早期 DM 患者的肌内膜毛细血管上有 MAC 沉积。在肌营养不良症中，非坏死纤维的肌膜上也观察到 MAC 沉积。分析炎症细胞、MHC I 类表达和 MAC 沉积有助于将肌营养不良症与特发性炎症性肌病区分开来。
