成人炎症性肌肉病的临床病理特点:诊断标准及分类的新认识

Clinical and pathological characteristics of adult idiopathic inflammatory myopathies: A new understanding of diagnostic criteria and classification

目的依据新的欧洲神经肌肉疾病中心和美国肌病研究协作组(ENMC)关于炎症性肌肉病的诊断标准和分类,总结成人炎症性肌肉病患者的临床、电生理和骨骼肌病理改变特点。方法回顾性分析北京大学第三医院53例成人炎症性肌肉病患者的临床、电生理特点,及肌肉、皮肤、神经活检的病理特点。结果 53例患者肌肉病理为肌源性损害伴炎性细胞浸润,其中,多发性肌炎19例,皮肌炎12例,包涵体肌炎6例,免疫介导性坏死性肌肉病4例,非特异性肌炎12例;其中5例患者伴发结缔组织病(系统性硬化症伴干燥综合征1例,系统性硬化症1例,干燥综合征3例),4例伴发恶性肿瘤(贲门癌2例,肺癌2例)。结论炎症性肌肉病的病理改变在全身骨骼肌中的分布是不一致的,在一块骨骼肌内的分布也是不一致的。故小的组织块可能错过典型的病理改变。ENMC诊断标准中的拟诊多发性肌炎、皮肌炎和包涵体肌炎,有助于早期诊断和治疗;ENMC诊断标准中新加入的免疫介导性坏死性肌肉病和非特异性肌炎均对临床诊治有利。ENMC标准更具科学性和临床实用性。

Objective To summarize the clinical features, electropbysiology and neuropathological characteristics of 53 cases of adult idiopathic inflammatory myopathy （IIM） based on the new diagnostic methods of Amato/ European Neuromuscular Centre Workshop （ENMC）. Methods The study was retrospectively analyzed the clinical, electrophysiology, neuropathological characteristics of 53 patients with adult IIM who underwent muscular biopsy, skin biopsy and nerve biopsy from January 2010 to September 2014. Results The neuropathological characteristics of muscular biopsy of 53 patients were myogenic damage and inflammatory cell infiltration. Classification according to ENMC criteria, 53 cases were classified into the following groups： polymyositis （n=19）, dermatomyositis （n=12）, inclusionbody myositis （n=6）, immune-mediated necrotizing myopathy （n=4）, nonspecific myositis （n= 12）. Among them 5 cases were accompanied with connective tissue diseases including systemic sclerosis accompanied with Sjogren syndrome （n=1）, systemic sclerosis （n=l）, Sjogren syndrome （n=3）; 4 cases were accompanied with malignant tumor, including gastric cardia cancer （n=2） and lung cancer （n=2）. Conclusion Pathological changes of IIM in the skeletal muscle is not consistent, even the distribution in a block in skeletal muscle is not consistent. So, little tissue masses may miss the typical pathological changes ENMC diagnostic criteria of suspected PM, suspected DM and suspected IBM, are helpful to early diagnosis and treatment. ENMC diagnosis standard of new IMNM and NSM is beneficial for clinical diagnosis and treatment,which is more scientific and feasible.