ReviewChronic respiratory disease
The similarities and differences between pleuroparenchymal fibroelastosis and idiopathic pulmonary fibrosis.
Review in Chronic respiratory disease. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
16 citing papers in PubMed, 25 citations in OpenAlex.
- Pleuroparenchymal Fibroelastosis in Connective Tissue Disease-Related Interstitial Lung Disease.Journal of clinical medicine · 2026Review
- A case of pleuroparenchymal fibroelastosis without radiological pleural involvement.BMC pulmonary medicine · 2026Article
- Rare interstitial lung diseases: a narrative review.Journal of thoracic disease · 2024Review
- 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
- Platythorax increases residual volume/total lung capacity in idiopathic pleuroparenchymal fibroelastosis.ERJ open research · 2024Article
- Proposed Clinical Algorithm for Pleuroparenchymal Fibroelastosis (PPFE).Journal of clinical medicine · 2024Review
- Evolutionary genetics of pulmonary anatomical adaptations in deep-diving cetaceans.BMC genomics · 2024Article
- Improvement of diaphragmatic excursion in the supine position in a patient with idiopathic pleuroparenchymal fibroelastosis: A case report.Respiratory medicine case reports · 2024Article
- Three cases of suspected pleuroparenchymal fibroelastosis after allogeneic hematopoietic cell transplantation.Blood cell therapy · 2023Article
- Impact of sleep-related hypoventilation in patients with pleuroparenchymal fibroelastosis.Respiratory research · 2022Article
- Determinants of Six-Minute Walk Distance in Idiopathic Pulmonary Fibrosis and Idiopathic Pleuroparenchymal Fibroelastosis.Biomedicines · 2022Article
- Prognostic and Clinical Value of Cluster Analysis in Idiopathic Pleuroparenchymal Fibroelastosis Phenotypes.Journal of clinical medicine · 2021Article
- The pathogenesis and pathology of idiopathic pleuroparenchymal fibroelastosis.Histology and histopathology · 2021Review
- Physiological Criteria Are Useful for the Diagnosis of Idiopathic Pleuroparenchymal Fibroelastosis.Journal of clinical medicine · 2020Article
- Rightward Shift of the QRS Axis in Idiopathic Pleuroparenchymal Fibroelastosis.Open respiratory archivesArticle
- Pleuroparenchymal Fibroelastosis. Is it Really Uncommon? Report of Eight Cases.Open respiratory archivesArticle
Corrections and comments
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Authors and funding
5 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The idiopathic form of pleuroparenchymal fibroelastosis (PPFE) is categorized as a rare idiopathic interstitial pneumonia in the current classification. The majority of PPFE cases are idiopathic, but many predisposing factors or comorbidities have been reported. Although histological PPFE is predominantly located in the upper lobes, which are less often affected by fibrosis in patients with idiopathic pulmonary fibrosis (IPF), the clinical course of PPFE is seemingly similar to that of IPF. However, upper lobe fibroelastosis has various clinical and physiological characteristics that differ from those of IPF, including a flattened thoracic cage and a marked decrease in the forced vital capacity (FVC) but with a preserved residual volume. Compared with IPF, the decrease in the walking distance is mild despite the markedly decreased FVC in PPFE, and chest radiograph more frequently shows the elevation of bilateral hilar opacities with or without tracheal deviation. The prognosis may be related to the development of fibrosing interstitial pneumonia in the lower lobes with elevated levels of serum Krebs von den Lungen-6; however, there is marked variation in the pathogenesis and clinical features in PPFE. A proposal of the diagnostic criteria for idiopathic PPFE with and without surgical lung biopsy, which has recently been published, may be useful.
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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.