Definition and research guide
This page combines a MeSH definition, publication statistics and selected research abstracts. It is a starting point for reading the literature, not a medical textbook or a systematic evidence review.
Content available: 3 complete abstracts (728 words), 9 references. Full article text is not stored or reproduced here; follow the PMID, DOI or PMC links to read the original sources and their access terms.
Definition
A process in which normal lung tissues are progressively replaced by fibroblasts and collagen causing an irreversible loss of the ability to transfer oxygen into the bloodstream via pulmonary alveoli. Patients show progressive dyspnea finally resulting in death.
Definition: NLM Medical Subject Headings (MeSH) scope note (Public domain (U.S. National Library of Medicine); attribution requested). View the MeSH record.
Also known as: Fibroses, Pulmonary; Fibrosis, Pulmonary; Pulmonary Fibroses; Alveolitis, Fibrosing; Alveolitides, Fibrosing; Fibrosing Alveolitides; Fibrosing Alveolitis; Idiopathic Diffuse Interstitial Pulmonary Fibrosis.
Where it fits in the MeSH classification
- Broader topics
- Lung Diseases, Interstitial; Fibrosis
- Narrower topics
- Idiopathic Pulmonary Fibrosis
Related concepts
- Fibroblasts
- Collagen
- Pulmonary Alveoli
- Dyspnea
Research landscape
PubMed indexes 32,778 records tagged with the MeSH heading “Pulmonary Fibrosis” (counts retrieved 2026-11-04). 9,267 of them (28.3%) were published from 2020 onward, and the busiest period so far is through 1999 with 11,143 records.
Reviews account for 4,491 records (13.7%) and meta-analyses for 211. Randomized controlled trials number 387 (1.2%) as a share of all records on the topic. Randomized trials make up only a small share of this literature, so much of what is known comes from laboratory, observational or descriptive work.
Show the data
| Period | Records |
|---|---|
| through 1999 | 11,143 |
| 2000-2009 | 4,129 |
| 2010-2019 | 8,239 |
| 2020 onward | 9,267 |
Publication-type chart data
| Type | Records | Share |
|---|---|---|
| Reviews | 4,491 | 13.7% |
| Meta-analyses | 211 | 0.6% |
| Randomized controlled trials | 387 | 1.2% |
Method: PubMed query "Pulmonary Fibrosis"[MeSH Terms] with publication-type and date filters, retrieved 2026-11-04.
Reading the evidence in respiratory medicine
Research on airway and lung disease mixes lung-function tests, symptom diaries, imaging and hospital records, so what was measured and in whom matters as much as the result. Many conditions here, such as asthma and COPD, vary between people and over time, and exposure studies (smoking, pollution, workplace dust) show association rather than proof of cause. Nothing on this page is advice about treatment or inhalers; anyone with breathing difficulty should seek care from a qualified clinician. Check how the condition was defined, the severity of the people studied and whether outcomes were symptoms, lung function or hospital admissions.
Evidence from open-access studies
These open-access studies come from PubMed’s best-match results for this heading (papers whose main topic it is), ranked here by citation count, with one recent addition. Each abstract is reproduced in full under its recorded open licence so that you can read the authors’ own words. They illustrate the literature; they are not a systematic summary of it. Citation counts measure attention, not reliability or clinical benefit.
| Study / authors | Year | Source label / design | Europe PMC citations | Licence / source |
|---|---|---|---|---|
| Deupirfenidone compared with pirfenidone and placebo in idiopathic pulmonary fibrosis (ELEVATE-IPF): a phase 2b randomized placebo-controlled trial | 2026 | Randomized controlled trial Design not recorded in structured metadata | 3 | cc by PMID 42085224 · DOI · PMC13424664 |
| Deep-Learning Algorithm Diagnostic Support for Usual Interstitial Pneumonia Pattern Recognition in Fibrotic Interstitial Lung Disease | 2026 | Research article Design not recorded in structured metadata | 3 | cc by PMID 41923409 · DOI · PMC13342441 |
| Sustained YAP/TAZ activation promotes aberrant alveolar epithelial cell differentiation and drives persistent fibrotic remodeling | 2026 | Research article Design not recorded in structured metadata | 2 | cc by PMID 42262884 · DOI · PMC13464018 |
Deupirfenidone compared with pirfenidone and placebo in idiopathic pulmonary fibrosis (ELEVATE-IPF): a phase 2b randomized placebo-controlled trial [1]
Abstract reading summary — source excerpts
- Opening excerpt
- RationaleDeupirfenidone is a strategically deuterated form of pirfenidone that retains pharmacodynamic activity but has a differentiated pharmacokinetic profile that may enable improved efficacy and favorable tolerability in patients with idiopathic pulmonary fibrosis (IPF).
- Closing excerpt
- gov number NCT05321420.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (265 words)
RationaleDeupirfenidone is a strategically deuterated form of pirfenidone that retains pharmacodynamic activity but has a differentiated pharmacokinetic profile that may enable improved efficacy and favorable tolerability in patients with idiopathic pulmonary fibrosis (IPF).ObjectivesTo evaluate the efficacy and safety of deupirfenidone compared with placebo and pirfenidone in patients with IPF.MethodsPatients were randomized 1:1:1:1 to deupirfenidone 550 mg TID, deupirfenidone 825 mg TID, pirfenidone 801 mg TID, or placebo. The primary endpoint was the rate of change in forced vital capacity (FVC) for the combined arms of deupirfenidone versus placebo at 26 weeks. The primary and secondary analyses used Bayesian and frequentist approaches, respectively.Measurements and main resultsA total of 257 patients with IPF were randomized, and the proportion on treatment at the end of the study was 80.0%, 68.3%, 64.6%, and 78.1% for the placebo, pirfenidone, deupirfenidone 550 mg, and deupirfenidone 825 mg arms, respectively. Posterior mean change in FVC for placebo was -110.71 mL (95% credible interval (CI), -148.75, -70.98), and for the combined deupirfenidone arms was -48.42 mL (95% CI, -87.66, -9.04) with a posterior mean difference of 62.29 mL (95% CI l, -6.13, 115.73; posterior probability, 0.985). Using a frequentist approach, the adjusted mean change in FVC for the placebo arm was -112.5 mL (95% CI, -167.2, -57.8), and for the deupirfenidone 825 mg arm was -21.5 mL (95% CI, -78.2, 35.1); the adjusted mean difference was 91.0 mL (95% CI, 12.2, 169.7; P = .02). The most common adverse events for each active treatment arm were gastrointestinal.ConclusionsIn patients with IPF, treatment with deupirfenidone slowed lung disease progression over 26 weeks.Trial registrationClinicaltrials.gov number NCT05321420.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 42085224 · DOI · PMC13424664
Deep-Learning Algorithm Diagnostic Support for Usual Interstitial Pneumonia Pattern Recognition in Fibrotic Interstitial Lung Disease [2]
Abstract reading summary — source excerpts
- Opening excerpt
- Background and objectiveHigh resolution computed tomography (HRCT) scan diagnostic classification for usual interstitial pneumonia (UIP) plays a critical role in therapeutic decision-making and clinical trial eligibility for interstitial lung disease (ILD) patients, but is subject to variability.
- Closing excerpt
- SOFIA may be a useful automated assistive tool to support improved diagnostic consistency.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (254 words)
Background and objectiveHigh resolution computed tomography (HRCT) scan diagnostic classification for usual interstitial pneumonia (UIP) plays a critical role in therapeutic decision-making and clinical trial eligibility for interstitial lung disease (ILD) patients, but is subject to variability. A deep learning algorithm, the Systematic Objective Fibrotic Imaging Analysis Algorithm (SOFIA), has been validated to assist classification of HRCTs based on current guidelines. In this study, we evaluate the impact of SOFIA on inter-observer agreement for UIP classification and prognostic accuracy of clinicians' assessment of ILD HRCTs.MethodsRadiologists and pulmonologists (reviewers) were invited to evaluate 203 HRCTs from a national fibrotic ILD registry, scoring each of four UIP categories (definite UIP, probable UIP, indeterminate, or alternative diagnosis). SOFIA outputs were then provided, and reviewers were able to reevaluate their scores. Changes in interobserver agreement for UIP classification and prognostic accuracy were calculated.ResultsThree hundred twelve reviewers (120 radiologists, 192 pulmonologists) from 49 countries evaluated 203 HRCT scans. Following SOFIA, inter-observer diagnostic agreement improved for definite UIP from moderate to good (ICCpre = 0.54[0.50-0.60]; ICCpost = 0.70[0.66-0.74]), and for probable UIP from fair to moderate (ICCpre = 0.30[0.27-0.35]; ICCpost = 0.53[0.49-0.58]). Following SOFIA, there was improved prognostic accuracy for reviewers' definite UIP, probable UIP, and indeterminate scores (significant change in c-index), and the proportion of reviewers whose probable UIP scores were significantly predictive of transplant-free survival increased by 42%.ConclusionProviding SOFIA algorithm output to clinicians reviewing HRCT scans improved diagnostic agreement and prognostic accuracy for fibrotic ILD. SOFIA may be a useful automated assistive tool to support improved diagnostic consistency.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 41923409 · DOI · PMC13342441
Sustained YAP/TAZ activation promotes aberrant alveolar epithelial cell differentiation and drives persistent fibrotic remodeling [3]
Abstract reading summary — source excerpts
- Opening excerpt
- YAP/TAZ signaling is required for initiation of lung alveolar repair, yet previous studies in idiopathic pulmonary fibrosis (IPF) predicted increased YAP/TAZ signaling in alveolar epithelial cells.
- Closing excerpt
- Blocking aberrant persistent YAP/TAZ activity promotes adaptive repair and has potential as a therapeutic strategy for pulmonary fibrosis.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (209 words)
YAP/TAZ signaling is required for initiation of lung alveolar repair, yet previous studies in idiopathic pulmonary fibrosis (IPF) predicted increased YAP/TAZ signaling in alveolar epithelial cells. We investigated whether persistent YAP/TAZ alveolar epithelial cell signaling contributes to failed epithelial repair and persistent fibrotic remodeling. In IPF lungs, we identified increased YAP+TAZ+ alveolar epithelial cells and increased transcriptional target expression. Pharmacological YAP/TAZ activation in human alveolar epithelial cell organoids and in murine AT2 cell organoids generated with genetic YAP/TAZ activation (YTactive) (via deletion of Hippo kinases Stk3 and Stk4) resulted in phenotype shifts into aberrant transitional and airway-like states. Bleomycin injury of YTactive mice resulted in persistent fibrotic remodeling at 28 and 56 days after bleomycin injury. Gene promoter activity associated with transitional cell markers (Krt19, Hopx, and Runx2) was increased in YTactive AT2 cells. Immunofluorescent staining showed a loss of AT2-associated Cebpa and increased Krt19 in YTactive lineage-traced AT2 cells 28 days after injury. Inhibition of YAP/TAZ using verteporfin resulted in improved lung repair in YTactive mouse lungs, including restored Cebpa and decreased Krt19+ transitional cells. These findings demonstrate that sustained YAP/TAZ activation drives abnormal alveolar repair and persistent fibrotic remodeling. Blocking aberrant persistent YAP/TAZ activity promotes adaptive repair and has potential as a therapeutic strategy for pulmonary fibrosis.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 42262884 · DOI · PMC13464018
Key reviews and meta-analyses
The most cited review-type papers among PubMed’s best-match results for this heading, listed with bibliographic details only. Follow the links for the full text and access terms.
- Research article Genome-wide association study identifies multiple susceptibility loci for pulmonary fibrosis [4]
- Research article Viral Infection Increases the Risk of Idiopathic Pulmonary Fibrosis: A Meta-Analysis [5]
- Research article Impact of Antifibrotic Therapy on Mortality and Acute Exacerbation in Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis [6]
- Research article Efficacy of antifibrotic drugs, nintedanib and pirfenidone, in treatment of progressive pulmonary fibrosis in both idiopathic pulmonary fibrosis (IPF) and non-IPF: a systematic review and meta-analysis [7]
- Research article Drug Treatment of Idiopathic Pulmonary Fibrosis: Systematic Review and Network Meta-Analysis [8]
- Research article Safety, tolerability and appropriate use of nintedanib in idiopathic pulmonary fibrosis [9]
References
- Maher TM, Hamblin MJ, Choi WI, Case AH, Tomos IP, Tzouvelekis AE, et al.. Deupirfenidone compared with pirfenidone and placebo in idiopathic pulmonary fibrosis (ELEVATE-IPF): a phase 2b randomized placebo-controlled trial. . 2026. PMID 42085224 · DOI · PMC13424664
- Fermoyle CC, Mackintosh JA, Navaratnam V, Ellis SJ, Cooper WA, Goh NSL, et al.. Deep-Learning Algorithm Diagnostic Support for Usual Interstitial Pneumonia Pattern Recognition in Fibrotic Interstitial Lung Disease. . 2026. PMID 41923409 · DOI · PMC13342441
- Gaona IP, McCall AS, Geis NM, Colvard AC, DiGiovanni GT, Sherrill TP, et al.. Sustained YAP/TAZ activation promotes aberrant alveolar epithelial cell differentiation and drives persistent fibrotic remodeling. . 2026. PMID 42262884 · DOI · PMC13464018
- Fingerlin TE, Murphy E, Zhang W, Peljto AL, Brown KK, Steele MP, et al.. Genome-wide association study identifies multiple susceptibility loci for pulmonary fibrosis. Nat Genet. 2013. PMID 23583980 · DOI · PMC3677861
- Sheng G, Chen P, Wei Y, Yue H, Chu J, Zhao J, et al.. Viral Infection Increases the Risk of Idiopathic Pulmonary Fibrosis: A Meta-Analysis. Chest. 2020. PMID 31730835 · DOI · PMC7214095
- Petnak T, Lertjitbanjong P, Thongprayoon C, Moua T. Impact of Antifibrotic Therapy on Mortality and Acute Exacerbation in Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis. Chest. 2021. PMID 34217681 · DOI
- Finnerty JP, Ponnuswamy A, Dutta P, Abdelaziz A, Kamil H. Efficacy of antifibrotic drugs, nintedanib and pirfenidone, in treatment of progressive pulmonary fibrosis in both idiopathic pulmonary fibrosis (IPF) and non-IPF: a systematic review and meta-analysis. BMC Pulm Med. 2021. PMID 34895203 · DOI · PMC8666028
- Canestaro WJ, Forrester SH, Raghu G, Ho L, Devine BE. Drug Treatment of Idiopathic Pulmonary Fibrosis: Systematic Review and Network Meta-Analysis. Chest. 2016. PMID 26836914 · DOI
- Corte T, Bonella F, Crestani B, Demedts MG, Richeldi L, Coeck C, et al.. Safety, tolerability and appropriate use of nintedanib in idiopathic pulmonary fibrosis. Respir Res. 2015. PMID 26400368 · DOI · PMC4581488
About this article
- Sources
- NLM Medical Subject Headings (definition, synonyms); NCBI PubMed (counts, identifiers); Europe PMC (open-access abstracts and citation counts).
- Verification
- Every reference was resolved against PubMed on 2026-11-04; title, year and DOI matched.
- Review state
- automated source verified; no human expert review has been recorded yet.
- Version
- 1, last updated 2026-11-04.
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Evidence strength
EvidenceHelix's computed view of the study designs behind this topic: how common meta-analyses, randomized trials and reviews are, how recent the literature is, and a plain-language reading. It describes the design mix of the literature. It is not a quality appraisal of any study, and it is not medical advice.