ArticleThe European respiratory journal2020
Pleuroparenchymal fibroelastosis in systemic sclerosis: prevalence and prognostic impact.
Article in The European respiratory journal, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers, 3 of them syntheses that pooled it.
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
23 citing papers in PubMed, 3 syntheses or guidelines pooled it, 54 citations in OpenAlex.
- The role of lung biopsy for diagnosis and prognosis of interstitial lung disease in systemic sclerosis: a systematic literature review.Respiratory research · 2024Pooled it
- Mediators of systemic sclerosis-associated interstitial lung disease (SSc-ILD): systematic review and meta-analyses.Thorax · 2023Pooled it
- Computed Tomography Predictors of Mortality or Disease Progression in Systemic Sclerosis-Interstitial Lung Disease: A Systematic Review.Frontiers in medicine · 2021Pooled it
- Quantitative assessment of fibroelastosis reveals distinct elastic and collagen fiber patterns in idiopathic and secondary pleuroparenchymal fibroelastosis.Virchows Archiv : an international journal of pathology · 2026Article
- 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
- Korean Guidelines for the Diagnosis and Management of Interstitial Lung Disease: Other Forms of Interstitial Lung Disease.Tuberculosis and respiratory diseases · 2025Article
- High resolution computed tomography in systemic sclerosis: From diagnosis to follow-up.Rheumatology and immunology research · 2024Article
- Proposed Clinical Algorithm for Pleuroparenchymal Fibroelastosis (PPFE).Journal of clinical medicine · 2024Review
- Pulmonary fibroelastosis - A review.Matrix biology : journal of the International Society for Matrix Biology · 2023Review
- 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
- Pleuroparenchymal fibroelastosis in rheumatoid arthritis-associated interstitial lung disease.Respiratory research · 2022Article
- Clinics in diagnostic imaging (212). Pleuroparenchymal fibroelastosis (PPFE).Singapore medical journal · 2022Article
- Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease.Turkish journal of medical sciences · 2022Article
- Demystifying idiopathic interstitial pneumonia: time for more etiology-focused nomenclature in interstitial lung disease.Expert review of respiratory medicine · 2022Review
- Radiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias.Respiratory research · 2021Article
- Pleuroparenchymal fibroelastosis in idiopathic pulmonary fibrosis: Survival analysis using visual and computer-based computed tomography assessment.EClinicalMedicine · 2021Article
- Lung Transplantation for Pleuroparenchymal Fibroelastosis.Journal of clinical medicine · 2021Review
- Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis.Journal of clinical medicine · 2021Article
Corrections and comments
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Authors and funding
23 authors at 11 institutions in 4 countries.
Funding
Abstract
Interstitial lung disease (ILD) in systemic sclerosis (SSc) is a major cause of morbidity and mortality, mostly presenting as non-specific interstitial pneumonia. Little is known about the prevalence of pleuroparenchymal fibroelastosis (PPFE), a specific entity affecting the visceral pleura and subpleural parenchyma. We set out to estimate PPFE prevalence in two large cohorts of SSc patients and to assess its impact on survival and functional decline.A total of 359 SSc patients, derived from two referral centres in two different countries (UK and Italy), were included. The first available high-resolution computed tomography scan was independently evaluated by two radiologists blind to clinical information, to quantify ILD extent, freestanding bronchial abnormalities, and lobar percentage involvement of PPFE on a four-point categorical scale. Discordant scores were adjudicated by a third scorer. PPFE extent was further classified as limited (≤2/18) or extensive (>2/18). Results were evaluated against functional decline and mortality.The overall prevalence of PPFE in the combined SSc population was 18% (11% with extensive PPFE), with no substantial difference between the two cohorts. PPFE was significantly linked to free-standing bronchial abnormalities (61%
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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.