Citation: Zheng, Z., Peng, F., Zhou, Y. (2024). Biomarkers in idiopathic pulmonary fibrosis: Current insight and future direction Biomarkers in idiopathic pulmonary fibrosis: Current insight and future direction. Chinese Medical Journal Pulmonary and Critical Care Medicine. https://doi.org/10.1016/j.eclinm.2021.101009
Abstract
Background: Idiopathic pulmonary fibrosis (IPF) and pleuroparenchymal fibroelastosis (PPFE) are known to
have poor outcomes but detailed examinations of prognostic significance of an association between these
morphologic processes are lacking.
Methods: Retrospective observational study of independent derivation and validation cohorts of IPF populations. Upper-lobe PPFE extent was scored visually (vPPFE) as categories of absent, moderate, marked. Computerised upper-zone PPFE extent (cPPFE) was examined continuously and using a threshold of 2¢5% pleural surface area. vPPFE and cPPFE were evaluated against 1-year FVC decline (estimated using mixed-effects models) and mortality. Multivariable models were adjusted for age, gender, smoking history, antifibrotic
treatment and diffusion capacity for carbon monoxide.
Findings: PPFE prevalence was 49% (derivation cohort, n = 142) and 72% (validation cohort, n = 145). vPPFE marginally contributed 314% to variance in interstitial lung disease (ILD) severity across both cohorts. In multivariable models, marked vPPFE was independently associated with 1-year FVC decline (derivation: regression coefficient 18¢3, 95 CI 8¢4728¢2%; validation: 7¢51, 1¢8513¢2%) and mortality (derivation: hazard ratio [HR] 7¢70, 95% CI 3¢5016¢9; validation: HR 3¢01, 1¢336¢81). Similarly, continuous and dichotomised cPPFE were associated with 1-year FVC decline and mortality (cPPFE 2¢5% derivation: HR 5¢26, 3¢009¢22; validation: HR 2¢06, 1¢283¢31). Individuals with cPPFE 2¢5% or marked vPPFE had the lowest median survival, the cPPFE threshold demonstrated greater discrimination of poor outcomes at two and three years than marked vPPFE.
Interpretation: PPFE quantification supports distinction of IPF patients with a worse outcome independent of established ILD severity measures. This has the potential to improve prognostic management and elucidate separate pathways of disease progression.
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