Pleuroparenchymal fibroelastosis in a UK tertiary ILD centre: determinants of radiological progression and survival

Pleuroparenchymal fibroelastosis (PPFE) is a rare form of interstitial lung disease (ILD). Some studies have shown that IPF patients with PPFE-like features have accelerated progression, poorer prognosis, and shorter telomeres. We examined the clinical, radiological, and physiological features of a UK PPFE cohort to identify the predictors of clinical outcomes.

A retrospective analysis was performed of consecutive patients with a multidisciplinary team (MDT) diagnosis of PPFE (February 2018 - 2026). A convenience sample of 70 consecutive IPF (Idiopathic pulmonary fibrosis) patients was used as a comparator cohort. Baseline demographics, complications, pulmonary function decline at 1-year, radiological progression, and mortality were assessed. Radiological progression was determined by visual scoring of serial CT scans. Kaplan-Meier survival analysis with log-rank testing compared survival between cohorts, and multivariable Cox regression identified predictors of PPFE radiological progression and mortality.

Forty-three patients had an MDT diagnosis of PPFE. Compared with IPF patients, those with PPFE were younger (71 vs 75 years), more frequently female (55.8% vs 15.7%), and more likely to be never-smokers (60.5% vs 18.6%). PPFE patients had lower BMI, lower FEV1, higher TLCO, and were more likely to have a history of recurrent infections, pneumothorax, or pneumomediastinum (all p<0.05), while FVC was comparable between groups. Co-existing ILD was present in 53% of PPFE patients, most commonly definite or probable UIP (65%). Among patients who underwent genetic testing, 88% (7/8) had a telomere biology-related gene variant. There were no significant differences in one-year lung function decline or overall survival between PPFE and IPF patients, irrespective of co-existing ILD. Baseline FVC% predicted independently predicted radiological progression, while age, baseline FVC% predicted, and change in FVC% predicted were independent predictors of mortality in PPFE.

PPFE exhibits a distinctive phenotype characterised by younger age, female predominance, low BMI and frequent pleural complications, survival was comparable to IPF. Baseline FVC emerged as a common predictor of both mortality and radiological progression , supporting its use as a pragmatic prognostic marker while highlighting the need for prospective validation.