皮肌炎的微血管免疫病理改变  

Immunopathological changes of micro-vessels in dermatomyositis

作　　者：沈光莉[1] 吕鹤[1] 毕鸿雁[1] 张巍[1] 姚生[1] 涂平[2] 袁云[1] 

机构地区：[1]北京大学第一医院神经内科,北京世纪坛医院神经内科100034 [2]北京大学第一医院皮肤科,100034

出　　处：《中华医学杂志》2006年第27期1912-1915,共4页National Medical Journal of China

摘　　要：目的探讨皮肌炎患者骨骼肌内微血管的免疫病理改变规律。方法对12例皮肌炎患者进行肌肉活检。选择10例非皮肌炎患者作为对照,进行常规组织学和酶组织化学和针对血管的免疫组织化学染色,第一抗体包括抗血管性血友病因子(vWF)抗体、抗血栓调节蛋白(TM)抗体和抗内皮细胞一氧化氮合酶(eNOS)抗体。结果所有患者出现束周分布的肌纤维变性和肌束衣内小血管周围不同程度的炎细胞浸润,非特异性酯酶染色显示肌纤维问毛细血管深染。免疫组化染色显示毛细血管的vWF、TM和eNOS在束周区域分布显著减少,肌束衣处的微小动脉和静脉的TM和eNOS也减少。结论皮肌炎的血管内皮细胞损害除毛细血管外,可以出现在其他微血管。TM以及eNOS在血管内皮细胞减少提示血管的抗凝和舒张功能下降。皮肌炎是一种弥漫性炎性血管内皮细胞病。Objective To investigate the immunopathological changes of micro-vessels in dermatomyositis. Methods Twelve patients with dermatomyositis, 5 males and 7 females, aged 40. 8 （6- 72） , underwent muscle biopsy of biceps muscle of arm （ n = 11 ） or deltoid muscle （ n = 1 ）. The specimens underwent routine histological, enzyme histochemical, and immunohistochemical staining and microscopy. Ten biopsy specimens from patients with other diseases were used as controls. Results Perifascicular atrophy of muscular fibers and inflammatory infiltration in perimysium were seen in all patients with dermatomyositis. Non-specific esterase staining showed deep staining of capillaries and micro vascular endothelium among the muscular fibers. Immunohistochemistry showed remarkable reduction of capillaries positive in von Willebrand factor （vWF）, thrornbomodulin （TM） , and endothelial cell nitric oxide synthase （eNOS） in the perifascicular region, and low expression of. eNOS and TM in the microvascular endothelium in the perimysium. Conclusion The vascular lesions of dermatomyositis are located not only in capillaries, but also in other microvessels. Lower expression of TM and eNOS in vascular endothelium suggests the reduction of anticoagulation and vasodilation functions of vascular endothelium. Dermatomyositis is an inflammatory vascular endothelial disease.

关 键 词：皮肌炎 血栓调节蛋白 一氧化氮合酶 

分 类 号：R593.2[医药卫生—内科学]