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. 2025 Mar 9;203(1):40.
doi: 10.1007/s00408-025-00797-4.

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

Hyun J Kim  1 , Jeremy M Weber  2 , Megan L Neely  2   3 , Amy Hajari Case  4 , Aiham H Jbeli  5 , Peide Li  6 , Amy L Olson  6 , Laurie D Snyder  2   3 ; IPF-PRO Registry investigators
Collaborators, Affiliations

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

Hyun J Kim et al. Lung. .

Abstract

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 % predicted, oxygen use and FVC % predicted facilitated the prediction of survival > 5 years. Understanding predictors of longer-term survival may facilitate conversations with patients about their prognosis and treatment.

Keywords: Interstitial lung diseases; Mortality; Observational study; Prognosis.

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Conflict of interest statement

Declarations. Competing interests: HJK is a principal investigator at a site in the IPF-PRO/ILD-PRO Registry. HJK has received research support from Boehringer Ingelheim, FibroGen, Galapagos, the National Institutes of Health, Roche/Genentech, United Therapeutics. JMW, MLN and LDS are employees of the Duke Clinical Research Institute, which receives funding support from Boehringer Ingelheim Pharmaceuticals, Inc to run the IPF-PRO/ILD-PRO Registry. JLT also reports grants from AstraZeneca and CareDx and participation in advisory boards for Altavant, Avalyn, Natera, Sanofi, Theravance. AHC is a principal investigator at a site in the IPF-PRO/ILD-PRO Registry. AHC has received research support from Boehringer Ingelheim, Roche/Genentech, United Therapeutics, FibroGen, Veracyte, Galapagos, Pilant, Kadmon, Bristol Myers Squibb, Bellerophon, Pulmonary Fibrosis Foundation (PFF), Galecto; consulting fees from Veracyte; speaker fees from Boehringer Ingelheim, Genentech, The France Foundation, Paradigm Medical; medical writing support from Boehringer Ingelheim, Veracyte, United Therapeutics; and has acted as a medical advisor to the PFF. AHJ has no disclosures. PL and ALO are employees of Boehringer Ingelheim Pharmaceuticals, Inc. Ethical Approval: The study was approved by the Duke University Institutional Review Board (Pro00046131). The protocol was approved by the relevant Institutional Review Boards and/or local Independent Ethics Committees prior to patient enrollment at every site (listed in the Acknowledgements). Consent to Participate: All patients provided written consent prior to entering the registry. Consent for Publication: Not applicable.

Figures

Fig. 1
Fig. 1
CART for survival based on variables at enrollment in the two-class survival model (≤ 5, > 5 years). * Time from first imaging evidence, symptoms, or diagnosis of IPF (whichever occurred first) to enrollment
Fig. 2
Fig. 2
CART for survival based on variables at enrollment and over the first year in the two-class survival model (≥ 1 to ≤ 5, > 5 years)
See this image and copyright information in PMC

References

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