What question did this study set out to answer?

This study aims to determine if the 2022 PPF criteria provide prognostic value for mortality in treated IPF patients.

July 18, 2026BMC Pulmonary Medicine0 citationsOpen Access

Progressive pulmonary fibrosis criteria predict mortality in treated idiopathic pulmonary fibrosis: a prospective cohort study

YWYu-Cheng WuNational Chung Hsing University CWC C WangTaichung Veterans General Hospital YSYi-Luen ShenAsia University

Key Points

This study aims to determine if the 2022 PPF criteria provide prognostic value for mortality in treated IPF patients.
Prospective cohort study of patients with confirmed IPF enrolled in the REGILD from 2018-2022.
Disease progression assessed one year post-antifibrotic therapy using the 2022 PPF criteria.
Cox regression and nested Cox model analyses evaluated predictors of mortality and model improvement.
Among 90 patients, 50 (55.6%) met the PPF criteria after one year of treatment.
Three-year mortality was significantly higher in those with PPF-defined progression (46.0%) compared to stable disease (12.5%, p = 0.001).
Lower BMI (HR 0.87, 95% CI 0.78–0.96, p = 0.006) and PPF-defined progression (HR 3.39, 95% CI 1.23–9.34, p = 0.018) were independently associated with increased mortality.

Abstract

The 2022 ATS/ERS/JRS/ALAT criteria for progressive pulmonary fibrosis (PPF) were developed for fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (IPF). Whether this multidimensional framework has prognostic value in patients with treated IPF remains uncertain. We evaluated the prevalence of PPF-defined progression and its association with mortality in patients with IPF receiving antifibrotic therapy. This prospective cohort study included patients with multidisciplinary discussion-confirmed IPF enrolled in the Registry of Interstitial Lung Disease (REGILD) between 2018 and 2022. Disease progression was assessed one year after initiation of antifibrotic therapy using the 2022 PPF criteria. The primary outcome was 3-year all-cause mortality. Cox regression and nested Cox model analyses were performed to identify prognostic factors and evaluate incremental prognostic value beyond the GAP score. Ninety patients were included, of whom 50 (55.6%) fulfilled PPF criteria after one year of antifibrotic therapy. During a median follow-up of 3.6 years, 28 patients (31.1%) died. Three-year mortality was significantly higher in patients with PPF-defined progression than in those with stable disease (46.0% vs. 12.5%, p = 0.001). In multivariable analysis, lower baseline body mass index (BMI) (HR 0.87, 95% CI 0.78–0.96, p = 0.006) and PPF-defined progression (HR 3.39, 95% CI 1.23–9.34, p = 0.018) were independently associated with mortality. Addition of PPF-defined progression to a model containing the GAP score significantly improved model fit (Δχ² = 5.610, p = 0.018). PPF-defined progression and lower baseline BMI were independently associated with mortality in patients with IPF receiving antifibrotic therapy. PPF-defined progression provided incremental prognostic information beyond the GAP score, suggesting that assessment of longitudinal disease trajectory may complement conventional baseline risk stratification in treated IPF.

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