Strand Matthew J, Sprunger David, Cosgrove Gregory P, Fernandez-Perez Evans R, Frankel Stephen K, Huie Tristan J, Olson Amy L, Solomon Joshua, Brown Kevin K, Swigris Jeffrey J
Division of Biostatistics, National Jewish Health, Denver, CO.
Autoimmune Lung Center and Interstitial Lung Disease Program, National Jewish Health, Denver, CO.
Chest. 2014 Sep;146(3):775-785. doi: 10.1378/chest.13-2388.
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.
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 (Dlco %), and we used both unadjusted and multivariable techniques to compare survival between these groups.
There were no significant differences between groups in longitudinal changes in FVC % or Dlco % 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 = .49 for difference; and the mean decrease in Dlco % 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 = .003) and certain multivariable analyses.
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.
肺损伤的普通型间质性肺炎（UIP）模式可能发生在结缔组织病（CTD）的背景下，但最常见于无已知病因的情况下，即在特发性肺纤维化（IPF）的临床背景中。我们的目的是观察和比较在CTD或IPF临床背景下经活检证实为UIP的患者之间肺功能和生存率的纵向变化。
我们使用纵向数据分析模型比较两组（IPF [n = 321]和CTD-UIP [n = 56]）的预测FVC百分比（FVC%）或预测肺一氧化碳弥散量百分比（Dlco%），并使用未调整和多变量技术比较这些组之间的生存率。
在诊断前或从诊断到之后10年，两组在FVC%或Dlco%的纵向变化方面无显著差异（在此期间，IPF组FVC%每年的平均下降幅度[95%CI]为4.1 [3.4, 4.9]，CTD-UIP组为3.5 [1.8, 5.1]，差异P = 0.49；IPF组Dlco%每年的平均下降幅度为4.7 [4.0, 5.3]，CTD-UIP组为4.3 [3.0, 5.6]，差异P = 0.60）。尽管肺功能无差异，但在未调整分析（对数秩检验P = 0.003）和某些多变量分析中，IPF患者的生存率较差。
尽管随着时间推移肺功能变化无显著差异，但CTD-UIP患者（至少是那些患有某些可分类CTD的患者）的生存期比IPF患者长——我们怀疑这一观察结果是由于IPF患者急性加重死亡率增加所致。
