Kojima S, Takagi A
Department of Neurology, Toranomon Hospital.
Rinsho Shinkeigaku. 1993 Jun;33(6):612-9.
Although major histocompatibility complex (MHC) class I molecules have been consistently demonstrated on muscle fibers of patients with inflammatory myopathies (IM), results concerning the expression of MHC class II molecules have been conflicting and mechanism of the expression remains to be elucidated. Expression of MHC and neural cell adhesion molecules (N-CAM) was analyzed in 38 cases of IM and in 13 cases of normal and disease controls by the immunocytochemical method. The sarcolemmaL HLA-ABC molecules were seen in all IM cases, and were visualized on the every muscle fiber in 68% of the cases. In contrast, the HLA-DR, -DP, or -DQ expression was seen on scattered fibers in 37%, 11%, 5% of IM cases, respectively. The HLA-DR reactivity was often seen on muscle fibers in the vicinity of infiltrating cells or on the perifascicular fibers. The HLA-DR positive fibers did not express N-CAM, suggesting a degenerating/regenerating process of muscle fibers was not contributory to the aberrant expression of MHC class II molecules. The perifascicular HLA-DR expression was related to the perifascicular atrophy and was more frequent in polymyositis complicated with interstitial pneumonitis. Those results suggest that increased expression of MHC class I molecules precede the aberrant expression of MHC class II molecules, and the perifascicular atrophy might reflect immunopathological processes.
尽管在炎症性肌病(IM)患者的肌纤维上一直都能检测到主要组织相容性复合体(MHC)I类分子,但关于MHC II类分子表达的结果却相互矛盾,其表达机制仍有待阐明。采用免疫细胞化学方法,对38例IM患者以及13例正常对照和疾病对照的MHC和神经细胞黏附分子(N-CAM)表达情况进行了分析。在所有IM病例中均可见肌膜上的HLA-ABC分子,68%的病例中每个肌纤维上都能观察到。相比之下,分别有37%、11%、5%的IM病例中,HLA-DR、-DP或-DQ表达见于散在的纤维。HLA-DR反应性常见于浸润细胞附近的肌纤维或束周纤维上。HLA-DR阳性纤维不表达N-CAM,提示肌纤维的退变/再生过程并非MHC II类分子异常表达的原因。束周HLA-DR表达与束周萎缩相关,在合并间质性肺炎的多发性肌炎中更为常见。这些结果表明,MHC I类分子表达增加先于MHC II类分子的异常表达,束周萎缩可能反映了免疫病理过程。