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. 2025 Oct 7:12:1679011.
doi: 10.3389/fmed.2025.1679011. eCollection 2025.

Changes in lung function and exercise capacity are strong predictors of mortality in patients with IPF receiving antifibrotic therapy

Ju Hyun Oh  1 , Moo Suk Park  2 , Man Pyo Chung  3 , Sung Hwan Jeong  4 , Jin Woo Song  5 , Sun Mi Choi  6 , Yong Hyun Kim  7 , Sung Woo Park  8 , Yangin Jegal  9 , Hee-Young Yoon  10 , Won-Il Choi  11 , Jung-Wan Yoo  12 , Hyun-Kyung Lee  13 , Sei-Hoon Yang  14 , Eun-Joo Lee  15 , Hye Sook Choi  16 , Hyung Koo Kang  17 , Jong Sun Park  18 , Jae Ha Lee  19
Affiliations

Changes in lung function and exercise capacity are strong predictors of mortality in patients with IPF receiving antifibrotic therapy

Ju Hyun Oh et al. Front Med (Lausanne). .

Abstract

Background: Idiopathic pulmonary fibrosis (IPF) is a chronic fibrosing interstitial pneumonia with poor prognosis. This study evaluated whether monitoring changes in lung function and exercise capacity during antifibrotic therapy offers superior prognostic value compared with baseline clinical parameters in IPF.

Methods: We retrospectively analyzed patients with IPF enrolled with the Korean IPF cohort registry between June 2016 and August 2021. Prognostic factors for mortality were assessed using Cox proportional hazards models and receiver operating characteristic (ROC) curve analysis.

Results: Among 1,229 patients (mean age 68.3 years; 82.8% male), 88.0% received antifibrotic therapy. During a median follow-up of 41.0 months, 37.9% of the treated patients died. Multivariable Cox analysis revealed that a decline in forced vital capacity (FVC) at 12 months, lower baseline diffusing capacity of the lungs for carbon monoxide (DLco), a decline in DLco at 12 months, and a reduction in the 6-min walk distance at 6 months, were independent risk factors for mortality in IPF patients receiving antifibrotic therapy. In the ROC curve analysis, the change in FVC at 12 months showed the highest predictive accuracy for mortality (area under the curve = 0.676; p < 0.001). Kaplan-Meier analysis demonstrated significantly poorer survival in patients with ≥5.8% decline in FVC and ≥11.5% decline in DLco over 12 months (p < 0.001 and p = 0.001, respectively).

Conclusion: Longitudinal changes in lung function and exercise capacity as indicators of response to antifibrotic therapy may serve as potential surrogate markers of mortality in patients with IPF.

Keywords: fibrosis; interstitial lung disease; lung function; mortality; prognosis.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision.

Figures

Figure 1
Figure 1
Changes in (a) FVC, (b) DLco and (c) 6MWD at 6 and 12 months according to survival status in patients with IPF receiving antifibrotics. FVC, forced vital capacity; DLco, diffusing capacity of lungs for carbon monoxide; 6MWD, six-minute walk distance. *Asterisks indicate statistically significant differences (p < 0.05) between non-survivors and survivors in the change from baseline to 6 or 12 months in FVC, DLco, and 6MWD.
Figure 2
Figure 2
ROC analysis of changes in pulmonary function and exercise capacity for predicting mortality in patients with IPF receiving antifibrotics. ROC, receiver operating characteristic; AUC, area under the curve; FVC, forced vital capacity; DLco, diffusing capacity of lungs for carbon monoxide; 6MWD, six-minute walk distance.
Figure 3
Figure 3
Kaplan–Meier survival curves according to 12-month changes in (a) FVC and (b) DLco in patients with IPF receiving antifibrotics. FVC, forced vital capacity; DLco, diffusing capacity of lungs for carbon monoxide.
See this image and copyright information in PMC

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