ArticleRespiratory research2020
Longitudinal functional changes with clinically significant radiographic progression in idiopathic pulmonary fibrosis: are we following the right parameters?
Article in Respiratory research, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT02632123 (Validation of the Risk Stratification Score in Idiopathic Pulmonary Fibrosis), which is not on this map. Cited by 21 papers, 1 of them a synthesis that pooled it.
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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.
Validation of the Risk Stratification Score in Idiopathic Pulmonary Fibrosis
Who cites it
21 citing papers in PubMed, 1 synthesis or guideline pooled it, 33 citations in OpenAlex.
- A systematic review of the role of quantitative CT in the prognostication and disease monitoring of interstitial lung disease.European respiratory review : an official journal of the European Respiratory Society · 2025Pooled it
- Assessment of fibrosing interstitial lung disease progression: CT visual score on radiological progressive pulmonary fibrosis criteria versus 3D-CT volume-histogram analysis.Japanese journal of radiology · 2026Article
- Distinct Metabolic Signatures Linked to High-Resolution Computed Tomography Radiographic Phenotypes in Stable and Progressive Fibrotic Lung Disease.Metabolites · 2026Article
- Progressive pulmonary fibrosis: the importance of identification and intervention.European respiratory review : an official journal of the European Respiratory Society · 2026Review
- Association of dual-energy CT-derived quantitative parameters with hydroxyproline levels and pulmonary function in nonspecific interstitial pneumonia.Frontiers in immunology · 2026Article
- Interstitial Lung Disease Outcome Prediction Using Quantitative Densitometry Indices on Baseline Chest Computed Tomography.Diagnostics (Basel, Switzerland) · 2025Article
- A combined measure of blood leukocytes, forced vital capacity and quantitative CT is highly predictive of mortality in IPF: results of a single-centre cohort study.BMC pulmonary medicine · 2025Article
- Identification of progressive pulmonary fibrosis: consensus findings from a modified Delphi study.Respiratory research · 2024Article
- Machine learning classifier is associated with mortality in interstitial lung disease: a retrospective validation study leveraging registry data.BMC pulmonary medicine · 2024Article
- Towards the adoption of quantitative computed tomography in the management of interstitial lung disease.European respiratory review : an official journal of the European Respiratory Society · 2024Review
- Histogram-based analysis in progressive pulmonary fibrosis: relationships between pulmonary functional tests and HRCT indexes.The British journal of radiology · 2023Article
- In vivo polarisation sensitive optical coherence tomography for fibrosis assessment in interstitial lung disease: a prospective, exploratory, observational study.BMJ open respiratory research · 2023Observational
- Delineating associations of progressive pleuroparenchymal fibroelastosis in patients with pulmonary fibrosis.ERJ open research · 2023Article
- Lung-function trajectories in COVID-19 survivors after discharge: A two-year longitudinal cohort study.EClinicalMedicine · 2022Article
- Digital quantification of p16-positive foci in fibrotic interstitial lung disease is associated with a phenotype of idiopathic pulmonary fibrosis with reduced survival.Respiratory research · 2022Article
- Early Radiographic Progression of Scleroderma: Lung Disease Predicts Long-term Mortality.Chest · 2022Article
- Radiographic Progression and Survival of the Different HRCT Patterns of Idiopathic Pulmonary Fibrosis.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2022Article
- Validation of the risk stratification score in idiopathic pulmonary fibrosis: study protocol of a prospective, multi-centre, observational, 3-year clinical trial.BMC pulmonary medicine · 2021Article
- Effect of genotype on the disease course in idiopathic pulmonary fibrosis despite antifibrotic treatment.Biomedical reports · 2021Article
- Radiological and Physiological Predictors of IPF Mortality.Medicina (Kaunas, Lithuania) · 2021Article
Corrections and comments
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Authors and funding
7 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundProgression of the disease in idiopathic pulmonary fibrosis (IPF) is difficult to predict, due to its variable and heterogenous course. The relationship between radiographic progression and functional decline in IPF is unclear. We sought to confirm that a simple HRCT fibrosis visual score is a reliable predictor of mortality in IPF, when longitudinally followed; and to ascertain which pulmonary functional variables best reflect clinically significant radiographic progression.
methodsOne-hundred-twenty-three consecutive patients with IPF from 2 centers were followed for an average of 3 years. Longitudinal changes of HRCT fibrosis scores, forced vital capacity (FVC), total lung capacity and diffusing lung capacity for carbon monoxide were considered. HRCTs were scored by 2 chest radiologists. The primary outcome was lung transplant (LTx)-free survival after the follow-up HRCT.
resultsDuring the follow-up period, 43 deaths and 11 LTx occurred. On average, the HRCT fibrosis score increased significantly, and a longitudinal increase > 7% predicted LTx-free survival significantly, with good specificity, but limited sensitivity. The correlation between radiographic and functional progression was moderately significant. HRCT progression and FVC decline predicted LTx-free survival independently and significantly, with better sensitivity, but worse specificity for a ≥ 5% decline of FVC. However, the area under the curve towards LTx-survival were only 0.61 and 0.62, respectively.
conclusionsThe HRCT fibrosis visual score is a reliable and responsive tool to detect clinically meaningful disease progression. Although no individual pulmonary function test closely reflects radiographic progression, a longitudinal FVC decline improves sensitivity in the detection of clinically significant disease progression. However, the accuracy of these methods remains limited, and better prognostication models need to be found.
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