Objective To compare the characteristics of connective tissue disease-associated pulmonary fibrosis(CTD-PF), undiffer-entiated connective tissue disease-associated pulmonary fibrosis( UCTD-PF) and idiopathic pulmonary fibrosis( IPF) . Methods Pa-tients with pulmonary fibrosis( PF) in Xijing Hospital from January 2015 to December 2015 were selected in this study,and the PF pa-tients with other known causes were excluded. The clinical manifestation, autoantibody serology, chest high resolution CT, blood gas a-nalysis and other characteristics were retrospectively analyzed. Results A total of 414 patients were enrolled in this study including 226 cases(54. 6%) of CTD-PF, 50 cases(12. 1%) of UCTD-PF and 138 cases (33. 3%) of IPF. The mean age of CTD-PF, UCTD-PF and IPF was 54. 4,57. 6 and 66. 8 years, respectively. The female accounted for 70. 8%,62. 0% and 21. 0% in CTD-PF, UCTD-PF and IPF patients, respectively. Smoking was more common in IPF patients than in CTD-PF, UCTD-PF patients. Varieties of autoanti-bodies were seen in CTD-PF group and UCTD-PF group, but the prevalence of each autoantibody in UCTD-PF group was fewer than in CTD-PF group. Only antinuclear antibody, anti-SSA antibody and anti-Ro-52 antibody were found in IPF group, and the other au-toantibodies were seldom seen in IPF. The inflammatory nodules were the common features in CTD-PF and UCTD-PF patients, while the honeycomb, pulmonary bulla and emphysema were the most frequent features in IPF group. In addition, the prevalences of pulmo-nary infection and respiratory failure were higher in IPF patients than in the other patients. Conclusion Patients with CTD-PF, UCTD-PF and IPF have some similar characteristics, but they show distinct features. The lung manifestation may be more serious in IPF patients than those in CTD-PF and UCTD-PF patients. Thus the overall assessment of PF, especially the autoantibodies monitor, will be conducive to the differential diagnosis of PF and proper treatment.%目的：比较结缔组织病相关肺纤维化( CTD-PF )、未分化结缔组织病相关肺纤维化( UCTD-PF )及特发性肺纤维化( IPF)临床特征。方法收集西京医院2015-01~2015-12诊断为肺纤维化的住院患者,同时排除已知其他原因导致的肺纤维化患者。回顾性分析三组患者临床表现、自身抗体、高分辨胸部CT、血气分析等特征。结果总共纳入414例患者,其中CTD-PF 226例(54．6%),UCTD-PF 50例(12．1%),IPF 138例(33．3%)。三组患者平均年龄分别为54．4岁,57．6岁和66．8岁,女性比率分别为70．8%,62．0%和21．0%。吸烟在IPF患者中更多见。 CTD-PF组自身抗体多种阳性,UCTD-PF组中亦可见到较多自身抗体阳性,但是UCTD-PF组各种自身抗体阳性的比率较CTD-PF组低。 IPF组可见ANA、抗SSA抗体和抗Ro-52抗体等阳性,其他较少见。 CTD-PF和UCTD-PF患者胸部CT以小结节影等炎性表现为主,IPF患者以蜂窝状影、肺大疱、肺气肿等纤维化表现多见。另外,IPF患者并发肺部感染和呼吸衰竭的比率显著高于CTD-PF和UCTD-PF患者。结论 CTD-PF、UCTD-PF、IPF三组患者临床特征有相似及重叠之处,但亦各有特点,IPF患者肺部表现更严重。因此,对PF患者常规进行系统评估尤其自身抗体监测,有助于不同PF的鉴别诊断及合理治疗。
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