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四例抗Ku抗体阳性特发性炎性肌病患者临床及病理分析

Anti-Ku antibody-positive idiopathic inflammatory myopathy:a clinical and pathological analysis of 4 cases
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摘要 目的探讨抗Ku抗体阳性特发性炎性肌病(IIM)患者的临床表现及骨骼肌病理特点。方法收集解放军总医院第一医学中心神经内科学部自2019年1月1日到2020年12月31日收治的4例抗Ku抗体阳性IIM患者资料进行回顾性分析,并结合国内外文献复习相关内容结果(1)本组4例患者发病年龄均偏大(41~68岁,平均57岁),均表现为四肢近端肌无力及不同程度的轴性肌群受累。(2)本组4例患者骨骼肌病理均可见肌细胞萎缩、坏死,肌间及小血管炎性细胞浸润等炎性肌病的改变,其中2例患者出现镶边空泡这一异常结构。结论抗Ku抗体阳性IIM患者四肢近端肌及轴性肌群常受累,骨骼肌病理可出现镶边空泡的异常结构。 Objective To investigate the clinical manifestations and skeletal muscle pathological features of patients with anti-Ku antibody positive idiopathic inflammatory myopathy(IIM).Methods The clinical data of 4 IIM patients with positive anti-Ku antibody,admitted to our hospital from January 1,2019 to December 31,2020,were retrospectively analyzed.The related literature at home and abroad were reviewed.Results(1)The IIM patients with positive anti-Ku antibody had onset at old age in this group(41-68 years old,mean 57 years old).The features of myasthenia included the involvement of the proximal muscles of the extremities and the axial muscle groups to varying degrees.(2)The changes of inflammatory myopathy,such as atrophy and degeneration of muscle cells,infiltration of inflammatory cells between muscles and small vessels,were observed in the skeletal muscle of the patients in this group;2 patients presented with abnormal structure of rimmed vacuoles.Conclusion The proximal muscles of the extremities and axial muscle groups can be involved in IIM patients with positive anti-Ku antibody,and skeletal muscle pathology can appear abnormal structure of rimmed vacuoles.
作者 陈娟 蔺颖 石强 Chen Juan;Lin Ying;Shi Qiang(Department of Neurology,First Medical Center of General Hospital of Chinese People's Liberation Army,Beijing 100853,China)
出处 《中华神经医学杂志》 CAS CSCD 北大核心 2021年第6期613-616,共4页 Chinese Journal of Neuromedicine
基金 国家自然科学基金面上项目(81771358)。
关键词 抗Ku抗体 肌炎 肌肉活检 病理染色 Anti-Ku antibody Myositis Muscle biopsy Pathological staining
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