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学科主题: 神经病学
题名:
抗信号识别颗粒抗体肌病八例临床分析
其他题名: Myopathy with antibodies to the signal recognition particle: report of 8 cases
作者: 王璐; 张巍; 郝洪军; 洪道俊; 高枫; 袁云
关键词: 信号识别颗粒 ; 肌炎 ; 生物医学研究 ; Signal recognition particle ; Myositis ; Biomedical research
刊名: 中华风湿病学杂志
发表日期: 2012
DOI: 10.3760/cma.j.issn.1007-7480.2012.09.004
卷: 16, 期:9, 页:593-595
收录类别: 中国科技核心期刊 ; 中文核心期刊 ; CSCD
文章类型: Journal Article
摘要: 目的 总结抗信号识别颗粒抗体肌病患者的临床和骨骼肌病理改变特点.方法 选择8例患者采取免疫印迹法测定血清肌炎抗体谱并进行肌肉活检,肌肉标本进行组织学、酶组织化学和免疫组织化学染色.免疫组织化学染色的第一抗体为鼠抗人CD8、CD20、CD68单克隆抗体,分别标记T细胞、B细胞和巨噬细胞,主要组织相容性复合体(MHC)-Ⅰ单克隆抗体标记肌纤维膜,CD31单克隆抗体标记毛细血管内皮细胞.结果 8例患者血清抗信号识别颗粒抗体均为强阳性,其中3例伴随Ro-52抗体阳性.8例患者骨骼肌出现肌纤维坏死、再生以及巨噬细胞浸润,2例患者伴随肌纤维肥大和间质增生,2例伴随少数T细胞浸润.存在部分MHC-Ⅰ阳性肌纤维,毛细血管密度正常.结论 抗信号识别颗粒抗体肌病以缓慢发病的肢体无力为主要表现,可伴肺部病变.肌纤维坏死、再生为其主要病理改变,可以类似肌营养不良改变. Objective We report the clinical and pathological features of 8 Chinese myopathy patients with antibodies to the signal recognition particle (SRP).Methods Serum myositis antibody profiles were tested with immunoblotting.Muscle biopsies were performed for histological,enzyme histochemical and immunohistochemical stainings.The first antibody in the immunohistochemical staining was mouse anti-human monoclonal antibodies including CD8,CD20,CD68,MHC- Ⅰ and CD31.Results Eight cases showed stark positive of anti SRP antibody,3 of them with positive anti Ro-52 antibody.The muscle biopsies showed necrotic and regenerative muscle fibers associated with infiltration of macrophage,but scattered T lymphocytes in 2 patients.Two of them presented with fiber hypertrophy and proliferation of connective tissue.There were some fibers with positive MHC-Ⅰ expression.Capillaries were almost normal.Conclusion The muscle weakness of myopathy with antibodies to SRP presents as a chronic progressive course and could associate with lung involvement.Fiber necrosis and regeneration are the main myopathological features,which can mimic muscular dystrophy in some cases.
语种: 中文
原文出处: 查看原文
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内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/41278
Appears in Collections:北京大学第一临床医学院_神经内科_期刊论文

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作者单位: 100034,北京大学第一医院神经内科
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