特发性炎性肌病肌肉病理特征与临床分型的关联性研究

Relationship between clinical classification and pathological features in patients with idiopathic inflammatory myopathies

目的:分析特发性炎性肌病(IIM)不同临床亚型的病理特征,研究IIM临床分型和病理分型的关系。方法:将2015年1月~2016年10月在中日友好医院住院的195例IIM患者,按临床分型分为皮肌炎(DM)、多发性肌炎(PM)、重叠性肌炎(OM)和肿瘤相关性肌炎(CAM)组,按肌肉病理特征分为病理型皮肌炎(pDM)、病理型多发性肌炎(pPM)、坏死性肌炎(NAM)、非特异性肌炎(USM)、MHC-I型肌炎(MHC-I)和正常组。分析患者的临床特征、临床型与病理型的关联性。结果:195例IIM患者中,DM、PM、OM和CAM分别占74.9%、14.9%、6.2%和4.1%;USM是所有IIM中最多见的病理类型,占34.9%,而PM中NAM病理分型最多见,占48.3%。NAM组患者的血清肌酶水平与其他病理分型组比较,均显著升高(均P<0.05)。MHC-I型组和正常组,肌无力发生的比例最低,正常组发生ILD的比例最少。结论:USM和NAM是IIM中常见的2种病理类型。通过肌活检病理特征对IIM进行分型,与患者的临床症状尤其是肌肉损伤程度关联性更强。

Objective：To analyze the pathological features of different clinical phenotypes of idiopathic inflammatory myopathies （IIM）,and to investigate the relationship between the clinieal classification and pathological features of IIM.Methods：Total 195 patients with IIM referred to China-Japan Friendship Hospital from Jan uary 2015 to October 2016 were divided into dermatomyositis （DM）,polymyositis （PM）,overlap myositis（OM） and cancer-associated myositis （CAM）as elinica＇s subsets by Bohan and Peter criteria.Pathologic subsets were classified as dermatomyositis （pDM）, pathologic polymyositis （pPM）, unspecified myositis （USM）, necrotizing my- opathy （NAM）,major histoeompatibility eomplex-I myositis （MHC-I）and normal group according to histopatho- logical features of muscle biopsy.Results：The frequency of DM,PM,OM and CAM among the 195 patients were 74.9%,14.9%,6.2% and 4.1% respeetively.USM with frequency of 34.9% was the most common patho- logical phenotype in all clinical subsets.NAM was more common in PM patients.Patients with NAM had the highest serum creatinine level within all pathologic groups （P〈0.05）.While patients in MHC-I and normal groups had a lower frequency of muscle weakness compared to other pathological groups.The frequency of ILD in normal pathological group was lowest in all groups.Conclusion：The most common pathological characteristic of IIM are USM and NAM.Pathological classification according to histopathological features of muscle biopsy is more related to clinical features in IIM patients.