摘要
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χ^2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DM65例，PM27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CADM2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（1．140，2854．885），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�
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 I1M 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 elinical course. Additionally,the decreased free triiodothyronine （FT3） and the elevation of tumor markers including carcino-emhryonic antigen （CEA）, neuron speeific 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 muhivariate 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%C1 （1.818, 548.093）, P=0.018] were po
出处
《中华风湿病学杂志》
CAS
CSCD
北大核心
2015年第12期805-812,共8页
Chinese Journal of Rheumatology
关键词
肌炎
肺疾病
间质性
变态反应和免疫学
Myositis
Lung disease, interstilial
Allergy and Immunology
