Clustering of clinical and CT biomarkers identifies reproducible prognostic subtypes in idiopathic PPFE

Integrated phenotyping of 69 patients with idiopathic pleuroparenchymal fibroelastosis (PPFE) using quantitative CT and clinical markers identified two reproducible clusters. The severe fibrotic phenotype carried an 8.6-fold higher mortality risk (95%CI 1.06-70.4). A four-variab…

• Retrospective design with an exploratory (n=32) and a validation (n=37) cohort.
• Age-adjusted leukocyte telomere length was not significantly shorter in PPFE vs controls, nor linked to survival, ruling out a major prognostic role.
• Key limitation: small sample size, single-country retrospective cohorts, and wide confidence intervals for the hazard ratio (1.06-70.4) indicating imprecise effect estimates.

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