ATS 2025: Baseline Characteristics and Survival Trends for Patients Diagnosed with Idiopathic Pulmonary Fibrosis Over 2016 – 2022

Introduction:

Idiopathic pulmonary fibrosis (IPF) has historically been associated with a median survival of 2–5 years, based on data preceding the introduction of antifibrotic therapies. This study was conducted to assess recent trends in patient characteristics and survival outcomes.

Methods:

A secondary analysis was performed using data from the prospective Canadian Registry for Pulmonary Fibrosis (CARE-PF). Patients diagnosed with IPF between 2016 and 2022 who had baseline pulmonary function tests within six months of diagnosis were included. Trends in mean age, forced vital capacity (FVC), and diffusing capacity for carbon monoxide (DLCO) were evaluated by year of diagnosis. Transplant-free survival was assessed using Kaplan-Meier estimates, and trends were tested using the Mann-Kendall method.

Results:

A total of 760 patients were included. The mean age at diagnosis increased from 69 years in 2016 to 72 years in 2022 (p = 0.003). The mean baseline FVC rose from 79% (95% CI 76–83%) to 85% (95% CI 80–90%) over the same period (p = 0.02). No significant trend was observed in DLCO, which remained between 53–61% (p = 0.37). The 3-year survival probability increased from 74% (95% CI 67–82%) in 2016 to 84% (95% CI 76–92%) in 2021, though this upward trend was not statistically significant (p = 0.06).

Conclusion:

From 2016 to 2022, increases in age and baseline FVC at the time of IPF diagnosis were observed. The 3-year survival remained generally above 70% and stable throughout the period, indicating better survival outcomes than historically reported.

Am J Respir Crit Care Med 2025; 211: A1732

American Thoracic Society 2025 International Conference, May 18-21, San Francisco