Idiopathic Pulmonary Fibrosis and Emphysema Decreased Survival Associated With Severe Pulmonary Arterial Hypertension
Idiopathic Pulmonary Fibrosis and Emphysema Decreased Survival Associated With Severe Pulmonary Arterial Hypertension
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DOI：
10.1378/chest.08-2306
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发表时间：
2009-07-01
期刊：
影响因子：
9.6
通讯作者：
Selman, Moises
中科院分区：
文献类型：
--
作者：
Mejia, Mayra;Carrillo, Guillermo;Selman, Moises
背景：肺气肿的存在改变了特发性肺纤维化（IPF）患者的预后。在这篇文章中，我们比较了临床特征，吸烟史，肺功能，估计收缩期肺动脉压（eSPAP），和死亡率在IPF肺气肿与IPF没有肺气肿的变化。从临床图表中收集临床数据。高分辨率CT（HRCT）扫描由对临床数据不知情的专家进行检查，患者分为以下两组：IPF伴肺气肿患者和IPF不伴肺气肿患者。Kaplan-Meier方法、log-rank检验和考克斯回归模型用于统计分析。结果：IPF队列中肺气肿的患病率为28%（110例患者中有31例）。IPF伴肺气肿与男性（比值比[OR]，18; 95%置信区间[CI]，2.7 - 773.7; p = 0.0003）和吸烟（OR，3.8; 95% CI，1.36 - 11.6; p = 0.004）显著相关。IPF和肺气肿患者在休息和运动期间的血氧饱和度平均（+/-SD）降低更高（分别为16.3 ± 6.7% vs 13.5 ± 4.6%; p = 0.04），平均纤维化HRCT扫描评分较高（分别为1.75 +/- 0.36 vs 1.55 +/- 0.38; p = 0.015），eSPAP较高（分别为82 +/- 20 vs 57 +/- 15 min Hg; p < 0.0001），中位生存时间较短（分别为25 vs 34个月; p = 0.01）。考克斯回归模型显示，与死亡率相关的两个最重要的变量是FVC < 50%预测值（风险比[HR]，2.6; 95% CI，1.19至5.68; p = 0.016）和eSPAP：75 min Hg（HR，2.25; 95%CI，1.12至4.54; p = 0.022）结论：与不伴肺气肿的IPF患者相比，伴肺气肿的IPF患者显示出更高的死亡率。这种可怕的预后似乎至少部分与严重肺动脉高压的发展有关。（CHEST 2009; 136：10-15）
Background: It has been suggested that the presence of emphysema modifies the outcome of patients with idiopathic pulmonary fibrosis (IPF). In this article we compare clinical features, smoking history, pulmonary function, estimated systolic pulmonary artery pressure (eSPAP), and mortality in IPF with emphysema vs IPF without emphysematous changes.Methods: A cohort of 110 IPF patients was evaluated. Clinical data were collected from clinical charts. High-resolution CT (HRCT) scans were examined by an expert blinded to clinical data, and patients were classified into the following two groups: patients with IPF with emphysema; and patients with IPF without emphysema. The Kaplan-Meier method, log-rank test, and Cox regression model were used for statistical analyses.Results: The prevalence of emphysema in the IPF cohort was 28% (31 of 110 patients). IPF with emphysema was significantly associated with male gender (odds ratio [OR], 18; 95% confidence interval [CI], 2.7 to 773.7; p = 0.0003), and smoking (OR, 3.8; 95% CI, 1.36 to 11.6; p = 0.004). Patients with IPF and emphysema had a higher mean ( +/-SD) decrease in oxygen saturation during rest and exercise (16.3 +/- 6.7% vs 13.5 +/- 4.6%, respectively; p = 0.04), a higher mean fibrosis HRCT scan score (1.75 +/- 0.36 vs 1.55 +/- 0.38, respectively; p = 0.015), a higher eSPAP (82 +/- 20 vs 57 +/- 15 min Hg, respectively; p < 0.0001), and lower median survival time (25 vs 34 months, respectively; p = 0.01) than patients with IPF without emphysema. The Cox regression model showed that the two most important variables associated with mortality were FVC < 50% predicted (hazard ratio [HR], 2.6; 95% CI, 1.19 to 5.68; p = 0.016) and eSPAP : 75 min Hg (HR, 2.25; 95% CI, 1.12 to 4.54; p = 0.022).Conclusions: IPF patients with emphysema exhibited higher mortality compared with those with IPF without emphysema. This dire prognosis seems to be at least partially associated with the development of severe pulmonary arterial hypertension. (CHEST 2009; 136:10-15)