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目的 探讨免疫机制在不同肌源性肌病病变过程中的作用。 方法 应用免疫组化 SP法系统观察 75例神经肌肉疾病患者和外科手术无肌病患者的正常骨骼肌细胞人类白细胞抗原 DR位点 (HL A- DR)的表达。 结果 HL A- DR定位于肌细胞膜 ,18例进行性肌营养不良症 (PMD)肌组织中 HL A - DR阳性表达 4例 (2 2 .2 % ) ,位于 HL A- DR阳性的单核细胞聚集区域 ,均为弱阳性 (+) ,15例多发性肌炎 (PM)和 10例皮肌炎 (DM)肌组织中肌细胞 HL A- DR阳性表达分别为 12例 (80 % )和 6例 (6 0 % ) (P <0 .0 5 ) ,不仅出现于炎症区域 ,远离炎症区也可见到 ,多为中阳性 ( )。代谢性肌病、先天性肌病、神经源性肌病和正常对照组肌细胞均未见 HL A- DR表达。 结论 除 PM/ DM等自身免疫性疾病有异常的免疫反应 ,其他类型的肌源性肌病特别是 PMD在病变过程中也有免疫因素的参与 ,可能对病变的进行性发展起一定的作用。
Objective To investigate the role of immune mechanism in the pathogenesis of different myogenic myopathy. Methods Immunohistochemical SP method was used to observe the expression of human leukocyte antigen DR (HL-DR) in normal skeletal muscle cells in 75 patients with neuromuscular diseases and surgical myopathy without myopathy. Results HL-DR localized in the muscle cell membrane, HL A - DR positive expression in muscle tissue of 18 patients with progressive muscular dystrophy (PMD) was found in 4 cases (22.2%), located in HL A- DR positive monocytes (+). The positive expression of HL-DR in muscle of 15 cases of polymyositis (PM) and 10 cases of dermatomyositis (DM) were 12 cases (80%) and 6 Cases (60%) (P <0. 05), not only in the inflammatory area, away from the inflammatory area can be seen, mostly in the positive (). Metabolic myopathy, congenital myopathy, neuromuscular myopathy and normal control group were not seen HL A-DR expression. Conclusion In addition to the abnormal immune response of autoimmune diseases such as PM / DM, other types of myogenic myopathy, especially PMD, have immune factors involved in the pathological process, which may play a role in the progressive development of the lesion.