ArticleRespiratory research2021
Radiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias.
Article in Respiratory research, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.
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Who cites it
8 citing papers in PubMed, 17 citations in OpenAlex.
- Pleuroparenchymal fibroelastosis-like findings identify an idiopathic pulmonary fibrosis subgroup with shortened telomeres and poor prognosis.ERJ open research · 2026Article
- Korean Guidelines for the Diagnosis and Management of Interstitial Lung Disease: Other Forms of Interstitial Lung Disease.Tuberculosis and respiratory diseases · 2025Article
- Neutrophil-lymphocyte ratio in patients with idiopathic pleuroparenchymal fibroelastosis.BMJ open respiratory research · 2023Article
- Pulmonary fibroelastosis - A review.Matrix biology : journal of the International Society for Matrix Biology · 2023Review
- Assessment of malnutrition-related risk in patients with idiopathic pleuroparenchymal fibroelastosis.ERJ open research · 2023Article
- 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
- Effects of Automatic Deep-Learning-Based Lung Analysis on Quantification of Interstitial Lung Disease: Correlation with Pulmonary Function Test Results and Prognosis.Diagnostics (Basel, Switzerland) · 2022Article
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Authors and funding
18 authors at 8 institutions in 1 country.
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Abstract
backgroundPleuroparenchymal fibroelastosis (PPFE) is characterised by predominant upper lobe pleural and subpleural lung parenchymal fibrosis. Radiological PPFE-like lesion has been associated with various types of interstitial lung diseases. However, the prevalence and clinical significance of radiological PPFE-like lesion in patients with idiopathic interstitial pneumonias (IIPs) are not fully understood. We aimed to determine the prevalence and clinical impact on survival of radiological PPFE-like lesion in patients with IIPs.
methodsA post-hoc analysis was conducted using data from the Japanese nationwide cloud-based database of patients with IIPs. All the patients in the database were diagnosed as having IIPs by multidisciplinary discussion. Patients diagnosed with idiopathic PPFE were excluded. Clinical data and chest computed tomography (CT) image of 419 patients with IIPs were analysed. The presence of radiological PPFE-like lesion was independently evaluated by two chest radiologists blind to the clinical data.
resultsOf the 419 patients with IIPs, radiological PPFE-like lesions were detected in 101 (24.1%) patients, mainly in idiopathic pulmonary fibrosis (IPF) and unclassifiable IIPs, but less in idiopathic nonspecific interstitial pneumonia. Prognostic analyses revealed that radiological PPFE-like lesion was significantly associated with poor outcome in patients with IIPs, which was independent of age, IPF diagnosis and %FVC. In survival analyses, the patients with radiological PPFE-like lesions had poor survival compared with those without (log-rank, p < 0.0001). Subgroup analyses demonstrated that radiological PPFE-like lesion was significantly associated with poor survival both in patients with IPF and those with unclassifiable IIPs.
conclusionRadiological PPFE-like lesion is a condition that could exist in IIPs, mainly in IPF and unclassifiable IIPs. Importantly, the radiological PPFE-like lesion is a non-invasive marker to predict poor outcome in patients with IIPs, which should be carefully considered in clinical practice.
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