Department of Medicine, University of British Columbia, Vancouver, BC, Canada.
Department of Radiology, Mayo Clinic, Rochester, MN.
Chest. 2013 Jul;144(1):234-240. doi: 10.1378/chest.12-2403.
Combined pulmonary fibrosis and emphysema (CPFE) is increasingly recognized, but its prevalence and prognosis remain unclear. We sought to determine the prevalence, clinical features, and prognosis of CPFE in idiopathic pulmonary fibrosis (IPF), using a standardized and reproducible definition.
Patients with IPF were identified from two ongoing cohorts. Two radiologists scored emphysema and fibrosis severity on high-resolution CT (HRCT) scans. CPFE was defined as ≥10% emphysema on HRCT scan. Clinical characteristics and outcomes of patients with CPFE and IPF and those with non-CPFE IPF were compared with unadjusted analysis and then analysis after adjustment for HRCT fibrosis score. Mortality was compared using competing risks regression to handle lung transplantation. Sensitivity analyses were performed using Cox proportional hazards, including time to death (transplantation censored) and time to death or transplant.
CPFE criteria were met in 29 of 365 patients with IPF (8%), with high agreement between radiologists (κ=0.74). Patients with CPFE had less fibrosis on HRCT scans and higher FVC, but greater oxygen requirements (P≤.01 for all comparisons). Findings were maintained with adjustment for fibrosis severity. Inhaled therapies for COPD were used by 53% of patients with CPFE. There was no significant difference in mortality comparing patients with CPFE and IPF to those with non-CPFE IPF (hazard ratio, 1.14; 95% CI, 0.61-2.13; P=.69).
CPFE was identified in 8% of patients with IPF and is a distinct, clinical phenotype with potential therapies that remain underutilized. Patients with CPFE and IPF and those with non-CPFE IPF have similar mortality.
肺纤维化合并肺气肿（CPFE）的发病率越来越高，但该病的流行率和预后仍不清楚。我们试图使用标准化和可重复的定义来确定特发性肺纤维化（IPF）中 CPFE 的流行率、临床特征和预后。
从两个正在进行的队列中确定了 IPF 患者。两名放射科医生对高分辨率 CT（HRCT）扫描的肺气肿和纤维化严重程度进行评分。CPFE 的定义为 HRCT 扫描中存在≥10%的肺气肿。使用未调整分析和调整 HRCT 纤维化评分后的分析比较了 CPFE 和 IPF 患者以及非 CPFE IPF 患者的临床特征和结局。使用竞争风险回归比较死亡率以处理肺移植。使用 Cox 比例风险进行了敏感性分析，包括死亡时间（移植截止）和死亡或移植时间。
CPFE 标准符合 365 例 IPF 患者中的 29 例（8%），放射科医生之间具有较高的一致性（κ=0.74）。CPFE 患者的 HRCT 扫描纤维化程度较低，FVC 较高，但氧气需求较高（所有比较 P≤0.01）。调整纤维化严重程度后仍存在这些发现。53%的 CPFE 患者使用了 COPD 的吸入疗法。CPFE 和 IPF 患者与非 CPFE IPF 患者的死亡率无显著差异（风险比，1.14；95%CI，0.61-2.13；P=0.69）。
CPFE 在 8%的 IPF 患者中被识别出来，这是一种具有潜在治疗方法的独特临床表型，但仍未得到充分利用。CPFE 和 IPF 患者与非 CPFE IPF 患者的死亡率相似。
