Predictors of Long-Term Survival in Patients with Idiopathic Pulmonary Fibrosis: Data from the IPF-PRO Registry

作者：Hyun Joo Kim, Jeremy M. Weber, Megan L. Neely, Amy Hajari Case, A. Jbeli, Peide Li, Amy L. Olson, Laurie D. Snyder, the IPF-PRO Registry investigators, Albert Baker, Scott Beegle, John A. Belperio, Rany Condos, Francis Cordova, Brian D. Southern, Daniel F. Dilling, John Fitzgerald, Kevin R. Flaherty, Kevin J. Gibson, Mridu Gulati, Kalpalatha K. Guntupalli, Nishant Gupta, David Hotchkin, Tristan J. Huie, Robert J. Kaner, Lisa Lancaster, Joseph A. Lasky, Doug Lee, Timothy Liesching, Randolph J. Lipchik, Jason Lobo, Tracy Luckhardt, Yolanda Mageto, M. Kokoszynska, Lake Morrison, Andrew M. Namen, Justin M. Oldham, Tessy Paul, David Zhang, Mary K. Porteous, Rishi Raj, Murali Ramaswamy, Tonya Russell, Paul Sachs, Zeenat Safdar, Shirin Shafazand, Ather Siddiqi, Reginald Fowler, Mary E. Strek, Hiram Rivas-Perez, Jeremy Tabak, Rajat Walia, Timothy P. Whelan · 发表于：Lung · 年份：2025 · DOI：10.1007/s00408-025-00797-4 · 被引用次数：8 · 研究领域：Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis、Systemic Sclerosis and Related Diseases、Chronic Obstructive Pulmonary Disease (COPD) Research

PURPOSE: We used data from the IPF-PRO Registry of patients with idiopathic pulmonary fibrosis (IPF) to identify characteristics that predicted survival for a further > 5 years. METHODS: Participants had IPF that was diagnosed or confirmed at the enrolling center in the previous 6 months. Patients were followed prospectively. A Classification And Regression Tree (CART) was used to identify predictors of survival > 5 versus ≤ 5 years following enrollment. The following variables, assessed at enrollment, were considered: age; body mass index (BMI); former smoker; current smoker; time from first imaging evidence, symptoms, or diagnosis of IPF to enrollment; forced vital capacity (FVC) % predicted; diffusing capacity of the lungs for carbon monoxide (DLco) % predicted; antifibrotic drug use; supplemental oxygen use; history of cardiac disease; pulmonary hypertension; COPD/emphysema; and rural location. RESULTS: The analysis cohort comprised 819 patients, of whom 278 (33.9%) survived > 5 years. DLco % predicted, supplemental oxygen use and FVC % predicted were the most important variables for predicting survival > 5 versus ≤ 5 years after enrollment. The importance of these variables (scaled such that the most important had an importance of 100%) was 100%, 78.2% and 74.2%, respectively. The optimism-corrected area under the curve (AUC) of the CART was 0.72, with an accuracy of 0.72. CONCLUSION: Among patients enrolled in the IPF-PRO Registry, a decision tree that included DLco % p...