炎症性肌病247例临床与病理研究

The clinical and pathological characteristics of 247 patients with infammatory myopathies

目的探讨多发性肌炎(PM)、间质性肌炎(IM)与神经肌炎(NM)的临床特点及神经肌肉活检的诊断价值。方法回顾分析247例炎症性肌病的临床表现及神经肌肉活检结果。结果3组临床表现相似,为近端肌无力、肌痛等;均有不同程度的肌酶增高,但PM组增高明显;IM和NM具有PM的病理改变,但炎性程度不及PM,又各有其特异性,IM组为炎性细胞浸润间质,NM组神经活检多有髓鞘脱失、炎性细胞浸润。结论PM、IM、NM炎性肌病的临床表现相似,诊断困难,需结合神经肌肉活检等辅助检查才能作出正确的诊断。

Objective To investigate the clinical and pathological characteristics of polymyositis （PM）, interstitial myositis （IM） and neuromyositis （NM） for its role to diagnosis. Methods The clinical features, muscular enzymes in serum, electromyogram （EMG） and neuromuscular biopsies of 247 patients with inflammatory myopathies were analyzed. Results The clinical manifestations in three groups were similar, characterized by the proximal myasthenia and myodynia. All the groups had seroenzyme increasing in different levels, whereas PM increased markedly. Pathologically, IM, and NM had less inflammatory infiltration than PM. Compared with PM, IM and NM had distinctive features in which the inflammatory cells infiltrate interstitial for IM and demyelination, inflammation of nerve for NM. Conclusions The clinical diagnosis of PM, IM, and NM was difficult as their similar manifestations. It is necessary to combine together seroenzyme test, EMG and neuromuscular biopsy for the definite diagnosis.