背景:先前的研究报道了结缔组织病(CTD)相关的常规间质性肺炎(UIP)和特发性肺纤维化(IPF/UIP)的生存率冲突。这项研究调查了诊断为CTD-UIP和IPF/UIP的患者的临床特征和预后的差异。
方法:完成了2008年1月至2013年6月IPF(n=88)和CTD-UIP(n=67)患者的回顾性回顾。我们比较了两组之间的人口统计学和临床特征。使用对数秩和Cox比例风险回归分析比较生存率。
结果:未分化CTD(UCTD)占已确诊CTD-UIP病例的33%。UCTD患者与CTD患者在人口学特征或生理参数方面差异均无统计学意义(均P>0.05)。然而,CTD-UIP患者更可能是年轻人,女性,与不吸烟的IPF/UIP组相比(均P<0.01)。IPF/UIP和CTD-UIP患者之间的生存率没有差异[风险比(HR),1.74;95%置信区间(CI),0.86-3.50;P=0.113]。然而,从CTD组移除UCTD患者显示IPF/UIP与CTD-UIP相比死亡风险增加2.47倍(HR,2.47;95%CI,1.01-6.09;P=0.049)。
结论:我们的研究结果表明,CTD-UIP患者的生存率与IPF/UIP患者相似。然而,UCTD似乎影响CTD-UIP患者的生存率。
BACKGROUND: Previous studies have reported conflicting survival rates for connective tissue disease (CTD)-associated usual interstitial pneumonia (UIP) and idiopathic pulmonary fibrosis (IPF/UIP). This study investigated the differences in the clinical characteristics and prognoses of patients diagnosed with CTD-UIP and IPF/UIP.
METHODS: A retrospective review of patients with IPF (n=88) and CTD-UIP (n=67) from January 2008 to June 2013 was completed. We compared the demographics and clinical characteristics between the two groups. Survival rates were compared using a log-rank and Cox proportional hazard regression analysis.
RESULTS: Undifferentiated-CTD (UCTD) accounted for 33% of the established CTD-UIP cases. No significant differences were identified in the demographic characteristics or physiological parameters between the UCTD and CTD patients (all P>0.05). However, the CTD-UIP patients were more likely to be young, female, and nonsmokers compared with the IPF/UIP group (all P<0.01). There was no difference in survival between the IPF/UIP and CTD-UIP patients [hazard ratio (HR), 1.74; 95% confidence interval (CI), 0.86-3.50; P=0.113]. However, the removal of the UCTD patients from the CTD group revealed that IPF/UIP was associated with a 2.47-fold increased risk of mortality compared with CTD-UIP (HR, 2.47; 95% CI, 1.01-6.09; P=0.049).
CONCLUSIONS: Our findings indicate that the survival of CTD-UIP patients was similar compared with that of IPF/UIP patients. However, it appears that UCTD influences the survival rate of CTD-UIP patients.