单纯多发性肌炎的临床、病理和CD28/CTLA-4:B7表达的研究

目的　研究单纯多发性肌炎 (SPM)的临床、病理特征和CD2 8/CTLA 4 :B7表达及其发病机制。方法　回顾性总结 14 1例SPM患者的临床资料 ,收集 6例治疗前症状高峰期PM病人的外周血 ,单色流式细胞术 (FCM)检测外周血淋巴细胞协同刺激分子CD2 8、CTLA 4、B7 1、BB 1和B7 2的表达 ,并与正常健康者对照。结果　本组主要表现肌无力、肌痛或肌捏痛 ,肌酸激酶 (CK)等血清肌酶谱增高 ,肌电图呈肌源性损害。肌肉病理主要表现为肌纤维变性坏死和再生 ,散在萎缩 ,肌内膜炎症细胞浸润。SPM组外周血淋巴细胞CD2 8、CTLA 4、B7 1、B7 2的表达增加 ,FCM显示CTLA 4及B7 1的平均荧光强度各自与对照组比较有显著性差异 (P <0 0 1) ,CD2 8及B7 2也有显著性差异 (P <0 0 5 ) ,BB 1在SPM组与对照组表达量均极少。结论　肌肉病理检查是诊断SPM的重要依据 ,协同刺激分子CD2 8/CTLA 4 :B7可能是SPM发病的重要环节。

Study on clinic, pathology and the expression of CD28/CTLA-4: B7 of simple polymyositis

Objective To study the clinical and pathological manifestation of simple polymyositis. To investigate the expression of costimulatory molecules CD28/CTLA-4: B7 in peripheral blood lymphocytes and their roles in the pathogenesis of SPM.Methods The clinical situation, serum emzymes, electromyography(EMG) and muscular pathology of 141 patients with SPM were investigated. The expression of costimulatory molecules CD28, CTLA-4, B7-1, BB-1 and B7-2 in peripheral blood lymphocytes of six patients with simple polymyositis was measured with one-color flow cytometry(FCM). The control group were healthy volunteers.Results Muscle weakless, myalgia, elevation of creatine kinase and abnormal EMG of myogenic damage were very frequently to see in SPM. The muscle biopsy showed degeneration, necrosis and regeneration of muscle fibres, sporadic muscle fibre atrophy and endomysial inflammatory infiltration. The expression levels of costimulatory molecules CD28, CTLA-4, B7-1 and B7-2 in peripheral blood lymphocytes increased in SPM. Compared to control group, the mean fluorescence intensity of these molecules in SPM group showed by FCM increased remarkably (CD28, B7-2, P<0.05; CTLA-4, B7-1,P<0.01). BB-1 was expressed less in peripheral blood lymphocytes either in SPM or normal control.Conclusion Muscular biopsy is a very important method in diagnosis of SPM. The costimulatory molecules CD28/CTLA-4: B7 may play an important role in the pathogenesis of SPM.