Pleuroparenchymal fibroelastosis-like findings identify an idiopathic pulmonary fibrosis subgroup with shortened telomeres and poor prognosis.

Authors

Ryota Otoshi, Hideya Kitamura, Takashi Niwa, Kota Murohashi, Tsuneyuki Oda, Tomohisa Baba, Eri Hagiwara, Tae Iwasawa, Tamiko Takemura, Koji Okudela, Yosui Nojima, Yoshinori Nakamura, Kenji Mizuguchi, Yayoi Natsume-Kitatani, Takashi Ogura

Published in

ERJ open research. Volume 12. Issue 4. Epub Jul 27, 2026.

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease (ILD) with poor prognosis. Radiological pleuroparenchymal fibroelastosis (PPFE)-like findings, characterised by upper-lobe subpleural fibrosis, have been associated with worse outcome in IPF. While short leukocyte telomere length (LTL) is a recognised prognostic factor, its relationship with PPFE-like findings remains unclear.
We conducted a secondary analysis of an ongoing ILD cohort. IPF patients who underwent LTL measurement by quantitative PCR were classified into those with PPFE-like findings on high-resolution computed tomography (IPF/PPFE group) and those without such findings (IPF/usual interstitial pneumonia (UIP) group). Clinical characteristics, pulmonary function, telomere length and outcomes were compared. Age-adjusted LTL was evaluated using healthy controls. Prognostic factors were analysed using Cox regression.
Among 179 IPF patients, 29 (16%) were assigned to the IPF/PPFE group. Compared to the IPF/UIP group (n=150), the IPF/PPFE group had lower body mass index and forced vital capacity, and significantly shorter LTL (p=0.002), with more patients below the 10th percentile of healthy controls (37.9% versus 12.0%). The IPF/PPFE group showed greater respiratory functional decline and higher mortality (65.5% versus 25.3%, p<0.001). In survival analysis, both PPFE-like findings and shortened LTL predicted worse outcomes; however, only PPFE-like findings remained independently associated with mortality in multivariate analysis.
IPF patients with PPFE-like findings constitute a distinct high-risk phenotype with shorter telomeres, accelerated progression, and poor prognosis. These findings highlight the clinical importance of recognising PPFE-like changes and telomere biology in IPF for risk stratification and emphasise the need for close monitoring and early intervention.

PMID:
42516906
Bibliographic data and abstract were imported from PubMed on 28 Jul 2026.

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