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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, electrophysiological and skeletal muscle pathological changes in adult patients with inflammatory muscle disease based on the diagnostic criteria and classification of inflammatory muscular disease in the new European Neuromuscular Disease Center and the American Society of Mycology Study Group (ENMC). Methods The clinical and electrophysiological characteristics of 53 patients with inflammatory muscle disease in the Third Hospital of Peking University were retrospectively analyzed. The pathological features of muscle, skin and nerve biopsy were retrospectively analyzed. Results The muscle pathology of 53 patients was myogenic damage accompanied by inflammatory cell infiltration. Among them, 19 cases of polymyositis, 12 cases of dermatomyositis, 6 cases of inclusion body myositis, 4 cases of immune-mediated necrotizing muscular disease, Non-myositis in 12 cases; 5 patients with connective tissue disease (systemic sclerosis with Sjogren’s syndrome in 1 case, systemic sclerosis in 1 case, Sjogren’s syndrome in 3 cases), 4 cases with malignant tumors (cardia 2 cases of cancer, 2 cases of lung cancer). Conclusion The distribution of pathological changes of inflammatory muscle disease in the whole body skeletal muscle is inconsistent, and the distribution in skeletal muscle is also inconsistent. Therefore, small organizations may miss the typical pathological changes. The diagnosis of polymyositis, dermatomyositis and inclusion body myositis in the diagnostic criteria of ENMC is helpful for early diagnosis and treatment. The newly added immunocompensational necrotizing muscle disease and non-myositis in ENMC diagnostic criteria Good for clinical diagnosis and treatment. ENMC standards more scientific and clinical practicality.