ReviewAnnals of the American Thoracic Society2019
Pleuroparenchymal Fibroelastosis. A Review of Clinical, Radiological, and Pathological Characteristics.
Review in Annals of the American Thoracic Society, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 76 papers.
What it found
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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.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
76 citing papers in PubMed, 170 citations in OpenAlex.
- A Multicenter Registry for Rare Interstitial Lung Diseases in Korea: Baseline Characteristics and Clinical Outcomes.Tuberculosis and respiratory diseases · 2026Article
- Pleuroparenchymal Fibroelastosis Lesion in Explanted Lungs of a Patient with Fibrotic Pulmonary Sarcoidosis.Internal medicine (Tokyo, Japan) · 2026Article
- Prevalence and outcomes of pleuroparenchymal fibroelastosis in patients with non-tuberculous mycobacterial pulmonary disease: a French retrospective cohort study.Journal of thoracic disease · 2026Article
- A Young Man With Apical Fibrotic Changes on Chest Imaging and History of Pneumothorax.CHEST pulmonary · 2026Article
- Upper lung asymmetry identifies distinct clinical phenotypes in idiopathic pleuroparenchymal fibroelastosis.BMC pulmonary medicine · 2026Article
- The pleuroparenchymal fibroelastosis atlas reveals aberrant cell states and their zonation as an alternate roadmap to lung fibrosis.Science advances · 2026Article
- Article
- 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
- Computed tomography imaging features of major pulmonary and extrapulmonary complications of fibrotic lung diseases.Einstein (Sao Paulo, Brazil) · 2026Review
- Article
- Multicentre retrospective observational study for development and validation of MAC prognostic score model.Scientific reports · 2025Observational
- Korean Guidelines for the Diagnosis and Management of Interstitial Lung Disease: Other Forms of Interstitial Lung Disease.Tuberculosis and respiratory diseases · 2025Article
- Retrospective Study of Clinical and Genetic Profiles of Alpha-Mannosidosis Patients From the UAE.JIMD reports · 2025Article
- A middle-aged man with dyspnea and hoarseness: an unusual case of vocal cord paralysis.BMC pulmonary medicine · 2025Article
- Rare interstitial lung diseases: a narrative review.Journal of thoracic disease · 2024Review
- The fissural involvement in pleuroparenchymal fibroelastosis: Something beyond the alveolar elastosis.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 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
- Platythorax increases residual volume/total lung capacity in idiopathic pleuroparenchymal fibroelastosis.ERJ open research · 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
16 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
7 authors at 1 institution in 1 country.
Funding
Abstract
Pleuroparenchymal fibroelastosis (PPFE) is an unusual pulmonary disease with unique clinical, radiological, and pathological characteristics. Designated a rare idiopathic interstitial pneumonia in 2013, its name refers to a combination of fibrosis involving the visceral pleura and fibroelastotic changes predominating in the subpleural lung parenchyma. Although a number of disease associations have been described, no single cause of PPFE has been unequivocally identified. A diagnosis of PPFE is most commonly achieved by identifying characteristic abnormalities on computed tomographic scans. The earliest changes are consistently located in the upper lobes close to the lung apices, the same locations where subsequent disease progression is also most conspicuous. When sufficiently severe, the disease leads to progressive volume loss of the upper lobes, which, in combination with decreased body mass, produces platythorax. Once regarded as a slowly progressing entity, it is now acknowledged that some patients with PPFE follow an inexorably progressive course that culminates in irreversible respiratory failure and early death. In the absence of effective medical drug treatment, lung transplant remains the only therapeutic option for this disorder. This review focuses on improving early disease recognition and evaluating its pathophysiological impact and discusses working approaches for its management.
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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.