Section of Pulmonary, Critical Care and Sleep Medicine, Department of Medicine, West Virginia University, Morgantown, WV, USA.
Human Studies Facility, US Environmental Protection Agency, Chapel Hill, NC, USA.
Int J Chron Obstruct Pulmon Dis. 2021 Jun 21;16:1873-1885. doi: 10.2147/COPD.S307192. eCollection 2021.
Disease heterogeneity in idiopathic pulmonary fibrosis (IPF) often complicates the systematic study of disease, management of patients and clinical investigations.
To describe combined pulmonary fibrosis emphysema (CPFE) phenotype in a rural Appalachian IPF cohort with the highest smoking rates in the United States.
CPFE patients (n = 60) in a developed IPF cohort (n = 153) were characterized. Groups (CPFE vs IPF without emphysema) were categorized based on the predominant HRCT patterns of UIP (n = 109). Demographics, clinical variables, and treatment details were recorded. Kaplan-Meier survival and multivariate logistic regression analysis were performed.
The prevalence of CPFE in our IPF cohort was 45% (n = 49). The CPFE group was younger (73.9 vs 78.2), had a more extensive smoking history (93.9% vs 53.3%) with greater mean smoking pack years (49.09 vs 15.39) and had lower percentage predicted DL on presentation (38.35 vs 51.09) compared to IPF without emphysema group. Both groups shared equivalent higher burden of comorbidities, including pulmonary hypertension (PH) (46.9% vs 33.3%). One-fifth of patients were prescribed antifibrotics and only a subset (5%) of patients underwent lung transplantation. There was a non-significant trend towards reduced survival in CPFE (p = 0.076). Smoking status and DL predicted CPFE in our cohort. Body mass index (BMI), PH, and pirfenidone use were significant predictors of mortality.
CPFE was highly prevalent in our rural IPF cohort. In contrast to previous studies, CPFE group was older and had higher female (approx. 30%) occurrence. A greater exposure to cigarette smoke and reduced DL at diagnosis predicted CPFE. Lower BMI and PH predicted higher mortality whereas use of pirfenidone improved survival in our cohort. This study highlights a complex interaction of cigarette smoking, advanced fibrosis of UIP, PH and potential utility of antifibrotic agents in CPFE phenotype. Substantial burden of comorbidities, older age, and the limited utilization of advanced therapeutics in the cohort emphasize the challenges faced by rural Appalachian patients.
特发性肺纤维化（IPF）的疾病异质性常常使疾病的系统研究、患者管理和临床研究复杂化。
描述美国吸烟率最高的阿巴拉契亚农村地区特发性肺纤维化（IPF）队列中合并性肺气肿（CPFE）表型。
对已建立的 IPF 队列（n=153）中的 CPFE 患者（n=60）进行了特征描述。根据 UIP 的主要 HRCT 模式（n=109），将各组（CPFE 与无肺气肿的 IPF）进行分类。记录了人口统计学、临床变量和治疗细节。进行了 Kaplan-Meier 生存分析和多变量逻辑回归分析。
在我们的 IPF 队列中，CPFE 的患病率为 45%（n=49）。CPFE 组年龄较小（73.9 岁 vs. 78.2 岁），吸烟史更广泛（93.9% vs. 53.3%），平均吸烟包年数更高（49.09 岁 vs. 15.39 岁），就诊时预测的 DL 百分比较低（38.35% vs. 51.09%）。与无肺气肿的 IPF 组相比，两组都有更高的合并症负担，包括肺动脉高压（PH）（46.9% vs. 33.3%）。1/5 的患者接受了抗纤维化治疗，只有一小部分（5%）的患者接受了肺移植。CPFE 组的生存率有下降的趋势，但无统计学意义（p=0.076）。吸烟状况和 DL 预测了我们队列中的 CPFE。体重指数（BMI）、PH 和吡非尼酮的使用是死亡的显著预测因子。
CPFE 在我们的农村 IPF 队列中患病率很高。与之前的研究不同，CPFE 组年龄较大，女性（约 30%）的发病率较高。更多的吸烟暴露和诊断时 DL 的降低预测了 CPFE。较低的 BMI 和 PH 预测了更高的死亡率，而在我们的队列中使用吡非尼酮改善了生存。本研究强调了吸烟、UIP 的晚期纤维化、PH 之间的复杂相互作用，以及抗纤维化药物在 CPFE 表型中的潜在效用。该队列中存在大量合并症、年龄较大以及对高级治疗方法的利用有限，这强调了农村阿巴拉契亚患者所面临的挑战。
