肌萎缩呈束周分布的青少年肌炎的临床与病理特点  

Clinical and muscle pathological study of 21 juvenile myositis patients with perifascicular atrophy changes

作　　者：刘智[1] 陈琳[1] 任海涛[1] 赵燕环[1] 

机构地区：[1]中国医学科学院北京协和医院神经科,100730

出　　处：《中华神经科杂志》2015年第9期776-780,共5页Chinese Journal of Neurology

摘　　要：目的 研究肌肉活体组织检查（简称活检）出现束周萎缩的青少年肌炎患者的临床及肌肉病理特点.方法 对21例肌肉活检病理以束周萎缩为病理特征的青少年肌炎患者的临床资料和病理改变进行回顾性分析.结果 临床表现：21例中11例确诊为青少年皮肌炎（典型皮疹）,9例为可能的青少年皮肌炎（不典型皮疹）,1例为混合结缔组织病.21例中19例有肌肉无力表现.9例肌酸激酶正常,4例轻度升高（3倍以下）,8例明显升高（3倍以上）,其中11例确诊的皮肌炎患者中10例肌酶正常或轻微升高.6例患者合并肺间质病变,11例抗核抗体阳性.2例合并EB病毒感染,1例巨细胞病毒感染.肌肉病理：21例均可见束周萎缩,其中4例束周萎缩形态学不典型,但相应束周肌纤维还原型辅酶、乳酸脱氢酶、细胞色素C氧化酶染色异常;12例免疫组织化学染色中9例显示膜补体攻击复合物染色阳性.比较分析发现肌酸激酶升高程度与肌纤维坏死相关,与束周萎缩无明显相关;膜补体攻击复合物沉积的标本中束周萎缩、局灶损伤及线粒体异常的病理改变更突出.结论 肌肉萎缩呈束周分布现象主要见于典型或不典型皮肌炎,可能与免疫复合物沉积和小血管异常有关.Objective To describe the clinical and muscle pathological characters of juvenile myositis patients with perifascicular atrophy （PFA）.Methods A series of 21 consecutive muscle biopsies with clinically and pathologically confirmed juvenile myositis were studied.All biopsies had PFA of muscle fibers.Results Clinical manifestation：11/21 had typical dermatomyositis rash,9/21 possible dermatomyositis because of atypical rash,1/21 mixed connective tissue disease;9/21 had weakness complaint;9/21with normal creatine kinase （CK） level,4/21 low grade rise,8/21 apparently elevated,10/ 11 definite dermatomyositis patients with normal or low grade level;6/21 combined with interstitial lung disease,2 with EB virus infection,1 with cytomegalo virus infection.Muscle pathology：4 specimens with atypical PFA also had enzyme abnormality in nicotinamide adenine dinucleotide,succinate dehydrogenase and cytochrome c oxidase staining,9/12 cases had membrane attack complement deposition intramuscular capillaries,and they had prominent regional mitochondrial abnormalities,protrude PFA and focal muscle damage.Skin biopsy of one case showed perivasculitis in dermis.Conclusions PFA could be seen in juvenile dermatomyositis and mixed connective tissue disease,and PFA could not increase the CK level.The capillary and intermediate-sized vessels damage may cause chronic ischemia,which could explain the PFA with mitochondrial abnormalities.

关 键 词：皮肌炎 肌萎缩 补体膜攻击复合物 线粒体 活组织检查 

分 类 号：R746.4[医药卫生—神经病学与精神病学]