摘要目的 分析出现束周萎缩的皮肌炎患者的临床病理特点.方法 回顾性分析2003-2011年北京协和医院神经肌肉病理实验室的肌肉病理资料,筛选出104例以束周萎缩为病理特点且临床资料完整的皮肌炎病例.结果 男性34例,女性70例,平均年龄45岁,其中8例肌电图未见异常,42例血清肌酶正常,75例患者合并间质性肺疾病.54例肌肉活检仅出现单纯束周萎缩,50例束周萎缩合并局灶损伤.单纯束周萎缩者与束周萎缩合并局灶损伤者间肌束膜小血管周围单个核炎性细胞浸润、膜攻击复合物在肌纤维间毛细血管的沉积有显著差异.12例患者同时行皮肤活检,其中6例符合典型交界性皮炎改变.结论 免疫介导的微小血管及中小血管病变引起的低灌注,可能参与束周萎缩及局灶肌纤维缺血的发生.出现束周萎缩的皮肌炎患者间质性肺疾病发生率高.

abstractsObjective To investigate the clinical and pathological characteristics of dermatomyositis with muscular perifascicular atrophy (PFA).Methods A series of 104 consecutive patients clinically and pathologically diagnosed as dermatomyositis by muscle biopsy in our laboratory from December,2003 to August,2011,were enrolled in this study. Muscle biopsy of all the enrolled patients had shown PFA of muscle fibers.Results Among the 104 patients,34 were males and 70 were females with a mean age of 45 years old.Among them,8 cases had normal electromyogram;42 had normal serum creatine kinase level;11 were diagnosed as carcinoma;75 were found to be combined with interstitial lung disease (ILD).Based on morphologic changes of muscle biopsy,they were divided into pure PFA group with 54 cases and PFA plus focal damage group with 50 cases.Compared with the pure PFA group,there was prominent mononuclear cell infiltration into perimysial intermediate sized vessels and membrane attack complement (MAC) deposition in the intramuscular capillaries in the PFA plus group.Skin biopsy had been taken in 12 cases together with muscle biopsy and had shown the "border effect"of both PFA and interface dermatitis in muscle and skin.Conclusions Our study suggests that chronic immune vascular damage and insufficiency in dermatomyositis may cause ischemia and focal myofiber damage in "watershed" regions. The incidence of ILD in our dermatomyositis patients with PFA is high.