ArticleEClinicalMedicine2021
Pleuroparenchymal fibroelastosis in idiopathic pulmonary fibrosis: Survival analysis using visual and computer-based computed tomography assessment.
Article in EClinicalMedicine, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers, 1 of them a synthesis that pooled it.
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Who cites it
12 citing papers in PubMed, 1 synthesis or guideline pooled it, 17 citations in OpenAlex.
- Comorbidities in clinical cohorts of patients with idiopathic pulmonary fibrosis: a systematic review and meta-analysis.European respiratory review : an official journal of the European Respiratory Society · 2026Pooled it
- Low-dose vs standard-dose photon-counting CT for myopathy-associated interstitial lung disease: a prospective feasibility study.European radiology · 2026Article
- The pleuroparenchymal fibroelastosis atlas reveals aberrant cell states and their zonation as an alternate roadmap to lung fibrosis.Science advances · 2026Article
- Korean Guidelines for the Diagnosis and Management of Interstitial Lung Disease: Other Forms of Interstitial Lung Disease.Tuberculosis and respiratory diseases · 2025Article
- Imaging technologies in experimental pulmonary fibrosis research: essential tool for enhanced translational relevance.European respiratory review : an official journal of the European Respiratory Society · 2025Review
- Proteomic profiling of bronchoalveolar lavage fluid uncovers protein clusters linked to survival in idiopathic forms of interstitial lung disease.ERJ open research · 2024Article
- Harnessing Advanced Machine Learning Techniques for Microscopic Vessel Segmentation in Pulmonary Fibrosis Using Novel Hierarchical Phase-Contrast Tomography Images.Methods of information in medicine · 2024Article
- Prognostic value of coexisting conditions and complications in pleuroparenchymal fibroelastosis: a single-center retrospective study.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2024Article
- Proteomic profiling of bronchoalveolar lavage fluid uncovers protein clusters linked to survival in idiopathic forms of interstitial lung disease.medRxiv : the preprint server for health sciences · 2024Article
- Imaging in the diagnosis and management of fibrosing interstitial lung diseases.Breathe (Sheffield, England) · 2024Review
- The world of rare interstitial lung diseases.European respiratory review : an official journal of the European Respiratory Society · 2023Review
- Delineating associations of progressive pleuroparenchymal fibroelastosis in patients with pulmonary fibrosis.ERJ open research · 2023Article
Corrections and comments
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Authors and funding
19 authors at 8 institutions in 3 countries.
Funding
Abstract
backgroundIdiopathic 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.
methodsRetrospective 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.
findingsPPFE prevalence was 49% (derivation cohort,
interpretationPPFE 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.
fundingThis research was funded in whole or in part by the Wellcome Trust [209,553/Z/17/Z] and the NIHR UCLH Biomedical Research Centre, UK.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.