炎性肌病合并急进性肺间质病变146例患者的发病及免疫学特征分析 ; The clinical and immunological feat ures of idiopathic inflammatory myopathy patients with rapid progressive interstitial lung disease

目的 研究炎性肌病(ⅡM)合并急进性肺间质病变的临床和免疫学特征,分析发生急进性肺间质病变的相关因素.方法 回顾1996年2月至2015年2月于北京大学人民医院住院的146例成人炎性肌病合并肺间质病变(ILD)患者.按DM、PM及临床无肌病性皮肌炎(CADM)分组,根据ILD是否呈急进性进行分层,分析炎性肌病各亚类合并急进性ILD的相关因素.离散型数据比较应用r检验,连续型数据应用独立样本t检验.结果 ①入组的146例合并ILD的炎性肌病患者包括DM 65例,PM 27例,CADM 54例,其中42.5％(62/146) ILD呈急进性病程.CADM患者急进性ILD发生率为55.6％,显著高于DM(35.4％)及PM(33.3％).②90％以上患者ILD同时或晚于炎性肌病发病,其中以DM组比例最高.③单因素相关分析显示对于DM组,同时或早于DM症状出现的ILD(早发ILD)、技工手、发热、氧分压降低、淋巴细胞下降、低白蛋白血症、α1球蛋白升高提示病变呈急进性;CADM组中合并吞咽肌无力的低肌病皮肌炎,CRP、α2球蛋白升高,肺活量下降及肺泡灌洗液中分叶核细胞比例升高与急进性ILD相关.而在DM及CADM 2组均可观察到肿瘤标记物包括癌胚抗原(CEA)、神经元特异性烯醇化酶(NSE)、细胞角蛋白19片段(CYFRA211)及铁蛋白水平与急进性ILD发生率呈正相关,且急进性ILD患者多合并低T3综合征(P＜0.05).④多因素回归分析显示技工手[HR=31.747,95％CI(2.367,425.817),P=0.009]为DM合并急进性ILD独立危险因素,CADM组为CEA[HR =57.047,95％CI(1.140,2 854.885),P=0.043]及CRP升高[HR=31.568,95％CI(1.818,548.093),P=0.018].抗Jo-1抗体阳性与急进性ILD无明显相关.结论 CADM患者中急进性ILD发生率高于DM及PM.ILD多同时或晚于炎性肌病发病,DM早期合并ILD易呈急进性.肿瘤标记物升高及低T3综合征提示ILD进展.技工手与CEA、CRP升高分别为DM及CADM合并急进性ILD的独立危险因素. ; Objective To investigate the clinical and immunological features of rapid progressive interstitial lung disease (ILD) in patients with idiopathic inflammatory myopathy (ⅡM).Methods The medical records of 146 adult ⅡM patients associated with ILD in Peking University People's Hospital from February 1996 to February 2015 were retrospectively analyzed.They were divided into three subgroups:the classic DM,PM and the clinical amyopathic dermatomyositis (CADM),and were further stratified based on with or without rapid progressive ILD (RP-ILD).Chi-squared test was used for group comparisons of categorical data and independent-sample t test for numerical data.Results ① Among 146 patients,62 (42.5％) developed RP-ILD.The prevalence of RP-ILD in CADM was signifcantly higher than dermatomyositis (DM) and polymyositis (PM).② ILD occurred after or at the same time with ⅡM in more than 90％ of patients.ILD arising before or simultaneously with DM (named "early-onset ILD") tended to progress rapidly.③ For the DM group,mechanic's hands,fever,lymphopenia,hypoxemia,hypoalbuminemia,and the elevation of globulin α1 were associated with RP-ILD.CADM-ILD with weakness of swallow muscles,increasing C-reactive protein (CRP) and globulin α2,decreased vital capacity and the relatively high level of segmented granulocytes in bronchoalveolar lavage fluid tended to undergo a rapid progressive clinical course.Additionally,the decreased free triiodothyronine (FT3) and the elevation of tumor markers including carcino-embryonic antigen (CEA),neuron specific enolase (NSE),cytokerantin-19-fragment (CYFRA211) and ferritin were significantly more frequent both in DM and CADM with RP-ILD (P＜0.05) patients.④ According to multivariate analysis,mechanic's hands [HR =31.747,95％CI (2.367,425.817),P=0.009] and the elevation of CEA [HR =57.047,95％CI (1.140,2 854.885),P=0.043] and CRP [HR=31.568,95％CI (1.818,548.093),P=0.018] were potential predictive factors for RP-ILD in DM and CADM patients,respectively.Conclusion The prevalence of RP-ILD in CADM is higher than DM and PM.ILD usually occurs after or simultaneously with ⅡM,early-onset ILD in DM patients tends to progressive rapidly.Increased tumor markers and decreased FT3 indicate the deterioration of disease.Mechanic's hands and the elevation of CEA and CRP are potential predictive factors for RP-ILD in DM and CADM patients,respectively. ; 中文核心期刊要目总览(PKU) ; 中国科技核心期刊(ISTIC) ; 中国科学引文数据库(CSCD) ; 12 ; 805-812 ; 19