Pulmonary Function and Survival in Idiopathic vs Secondary Usual Interstitial Pneumonia
Pulmonary Function and Survival in Idiopathic vs Secondary Usual Interstitial Pneumonia
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DOI：
10.1378/chest.13-2388
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发表时间：
2014-09-01
期刊：
影响因子：
9.6
通讯作者：
Swigris, Jeffrey J.
中科院分区：
文献类型：
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作者：
Strand, Matthew J.;Sprunger, David;Swigris, Jeffrey J.
背景：通常的间质性肺炎（UIP）肺损伤模式可能发生在结缔组织病（CTD）的背景下，但在特发性肺纤维化（IPF）的临床背景下，它最常见于病因不明的情况。我们的目的是观察和比较在CTD或IPF的临床背景下活检证实的UIP患者的肺功能和生存率的纵向变化。方法：我们使用纵向数据分析模型比较各组（IPF [n = 321]和CTD-UIP [n = 56]）预测FVC （FVC %）或预测肺一氧化碳弥散能力（D-LCO %）的百分比，我们使用未调整和多变量技术比较这些组之间的生存率。结果:各组之间没有明显差异的纵向变化FVC %或D-LCO %诊断,或从诊断到10年以外(在这段时间,平均每年减少FVC %(95%置信区间)是4.1 [3.4,4.9]IPF CTD-UIP和3.5 [1.8,5.1],P 5.49区别;和平均降低D (LCO) %每年4.7 [4.0,5.3]IPF CTD-UIP和4.3 [3.0,5.6],P = .60区别)。尽管肺功能没有差异，但在未调整（log-rank P 5.003）和某些多变量分析中，IPF患者的生存率较差。结论：尽管肺功能随时间的变化无显著差异，但CTD-UIP患者（至少具有某些可分类ctd的患者）比IPF患者活得更长，我们怀疑这一观察结果是由于IPF患者致命急性加重的发生率增加。
BACKGROUND: The usual interstitial pneumonia (UIP) pattern of lung injury may occur in the setting of connective tissue disease (CTD), but it is most commonly found in the absence of a known cause, in the clinical context of idiopathic pulmonary fibrosis (IPF). Our objective was to observe and compare longitudinal changes in pulmonary function and survival between patients with biopsy-proven UIP found in the clinical context of either CTD or IPF.METHODS: We used longitudinal data analytic models to compare groups (IPF [n = 321] and CTD-UIP [n = 56]) on % predicted FVC (FVC %) or % predicted diffusing capacity of the lung for carbon monoxide (D-LCO %), and we used both unadjusted and multivariable techniques to compare survival between these groups.RESULTS: There were no significant differences between groups in longitudinal changes in FVC % or D-LCO % up to diagnosis, or from diagnosis to 10 years beyond (over which time, the mean decrease in FVC % per year [95% CI] was 4.1 [3.4, 4.9] for IPF and 3.5 [1.8, 5.1] for CTD-UIP, P 5.49 for difference; and the mean decrease in D (LCO) % per year was 4.7 [4.0, 5.3] for IPF and 4.3 [3.0, 5.6] for CTD-UIP, P =.60 for difference). Despite the lack of differences in pulmonary function, subjects with IPF had worse survival in unadjusted (log-rank P 5.003) and certain multivariable analyses.CONCLUSIONS: Despite no significant differences in changes in pulmonary function over time, patients with CTD-UIP (at least those with certain classifiable CTDs) live longer than patients with IPF-an observation that we suspect is due to an increased rate of mortal acute exacerbations in patients with IPF.