In Brief | Transplant Medicine & Pulmonology
Transplant Medicine: Progressive Pulmonary Fibrosis Carries a Prognosis Similar to IPF in Lung Transplant Candidates
[30 June 2026]
Study Snapshot
| Design | Retrospective cohort study |
| Population | 194 adults with fibrotic interstitial lung disease listed for lung transplantation: 51 IPF, 121 PPF, 22 non-PPF fibrotic ILD |
| Comparison | Transplant-free survival and waiting-list mortality compared across IPF, PPF, and non-PPF groups; PPFE subtype analysed separately |
| Primary Outcome | Transplant-free survival: PPF vs non-PPF, P<0.001; PPF vs IPF, P=0.132 (comparable). Waiting-list mortality: PPF vs non-PPF, P=0.001; PPFE vs IPF, P=0.019 (significantly higher) |
Summary
Interstitial lung disease encompasses a heterogeneous group of conditions with variable trajectories, and the prognosis of lung transplant candidates with progressive pulmonary fibrosis (PPF) — a phenotype defined by an IPF-like disease course — has remained poorly characterised, creating uncertainty around optimal referral and listing timing.
This retrospective cohort study compared 194 adults with fibrotic ILD listed for lung transplantation across three groups: IPF, PPF, and non-PPF fibrotic ILD. Patients with PPF presented with more advanced physiological impairment than those with IPF, including lower mean forced vital capacity (47.0 ± 16.2%) and shorter six-minute walk distances (median 310 m).
Despite the heterogeneity within the PPF group, transplant-free survival and waiting-list mortality in PPF closely tracked IPF and were significantly worse than non-PPF disease. Patients with pleuroparenchymal fibroelastosis (PPFE), a PPF subtype, fared particularly poorly, with significantly higher waiting-list mortality than IPF.
These findings support treating PPF — and PPFE in particular — with the same urgency as IPF when considering transplant referral and listing, rather than waiting for further clinical deterioration.
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