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: 4 complete abstracts (604 words), 10 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 common interstitial lung disease of unknown etiology, usually occurring between 50-70 years of age. Clinically, it is characterized by an insidious onset of breathlessness with exertion and a nonproductive cough, leading to progressive dyspnea. Pathological features show scant interstitial inflammation, patchy collagen fibrosis, prominent fibroblast proliferation foci, and microscopic honeycomb change.
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: Idiopathic Pulmonary Fibroses; Pulmonary Fibroses, Idiopathic; Cryptogenic Fibrosing Alveolitis; Cryptogenic Fibrosing Alveolitides; Fibrosing Alveolitides, Cryptogenic; Pulmonary Fibrosis, Idiopathic; Fibrosing Alveolitis, Cryptogenic; Fibrocystic Pulmonary Dysplasia.
Where it fits in the MeSH classification
- Broader topics
- Pulmonary Fibrosis
- Narrower topics
- Hamman-Rich Syndrome; Idiopathic Interstitial Pneumonias
Related concepts
- Dyspnea
Research landscape
PubMed indexes 10,387 records tagged with the MeSH heading “Idiopathic Pulmonary Fibrosis” (counts retrieved 2026-11-04). 5,146 of them (49.5%) were published from 2020 onward, and the busiest period so far is 2020 onward with 5,146 records.
Reviews account for 1,856 records (17.9%) and meta-analyses for 163. Randomized controlled trials number 240 (2.3%) as a share of all records on the topic.
Show the data
| Period | Records |
|---|---|
| through 1999 | 119 |
| 2000-2009 | 684 |
| 2010-2019 | 4,438 |
| 2020 onward | 5,146 |
Publication-type chart data
| Type | Records | Share |
|---|---|---|
| Reviews | 1,856 | 17.9% |
| Meta-analyses | 163 | 1.6% |
| Randomized controlled trials | 240 | 2.3% |
Method: PubMed query "Idiopathic 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 |
|---|---|---|---|---|
| Inhibition of epithelial cell YAP-TEAD/LOX signaling attenuates pulmonary fibrosis in preclinical models | 2025 | Research article Design not recorded in structured metadata | 35 | cc by PMID 40753090 · DOI · PMC12318044 |
| Myeloid-mesenchymal crosstalk drives ARG1-dependent profibrotic metabolism via ornithine in lung fibrosis | 2025 | Research article Design not recorded in structured metadata | 22 | cc by PMID 40875483 · DOI · PMC12578403 |
| Impaired AMPK control of alveolar epithelial cell metabolism promotes pulmonary fibrosis | 2025 | Research article Design not recorded in structured metadata | 22 | cc by PMID 40591409 · DOI · PMC12333953 |
| Human iPSC-based Modeling of Pulmonary Fibrosis Reveals p300/CBP Inhibition Suppresses Alveolar Transitional Cell State | 2026 | Research article Design not recorded in structured metadata | 4 | cc by PMID 41680175 · DOI · PMC12901050 |
Inhibition of epithelial cell YAP-TEAD/LOX signaling attenuates pulmonary fibrosis in preclinical models [1]
Abstract reading summary — source excerpts
- Opening excerpt
- Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal disease characterized by excessive extracellular matrix deposition.
- Closing excerpt
- We thus identify YAP-TEAD/LOX inhibition in alveolar type II cells as a promising potential therapy for IPF patients.
- 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 (132 words)
Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal disease characterized by excessive extracellular matrix deposition. Current IPF therapies slow disease progression but do not stop or reverse it. The (myo)fibroblasts are thought to be the main cellular contributors to excessive extracellular matrix production in IPF. Here we show that fibrotic alveolar type II cells regulate production and crosslinking of extracellular matrix via the co-transcriptional activator YAP. YAP leads to increased expression of Lysl oxidase (LOX) and subsequent LOX-mediated crosslinking by fibrotic alveolar type II cells. Pharmacological YAP inhibition via verteporfin reverses fibrotic alveolar type II cell reprogramming and LOX expression in experimental lung fibrosis in vivo and in human fibrotic tissue ex vivo. We thus identify YAP-TEAD/LOX inhibition in alveolar type II cells as a promising potential therapy for IPF patients.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 40753090 · DOI · PMC12318044
Myeloid-mesenchymal crosstalk drives ARG1-dependent profibrotic metabolism via ornithine in lung fibrosis [2]
Abstract reading summary — source excerpts
- Opening excerpt
- Idiopathic pulmonary fibrosis (IPF) is a disease of progressive lung remodeling and collagen deposition that leads to respiratory failure.
- Closing excerpt
- Taken together, our findings define an immune-mesenchymal circuit that governs profibrotic metabolism in lung 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 (171 words)
Idiopathic pulmonary fibrosis (IPF) is a disease of progressive lung remodeling and collagen deposition that leads to respiratory failure. Myeloid cells are abundant in IPF lung and in murine lung fibrosis, but their functional effects are incompletely understood. Using mouse and human lung models, we show that ornithine produced by myeloid cells expressing arginase 1 (ARG1) serves as a substrate for proline and collagen synthesis by lung fibroblasts. The predominant ARG1-expressing myeloid cells in mouse lung were macrophages, but in IPF lung, high-dimensional imaging revealed ARG1 was expressed mainly in neutrophils. Small-molecule ARG1 inhibition suppressed both ornithine levels and collagen expression in cultured, precision-cut IPF lung slices and in murine lung fibrosis. These results were confirmed in macrophage-specific Arg1-KO mice. Furthermore, we found that this pathway is regulated by cell-to-cell crosstalk, starting with purinergic signaling: extracellular ATP receptor P2RX4 was necessary for fibroblast IL-6 expression, which, in turn, was necessary for ARG1 expression by myeloid cells. Taken together, our findings define an immune-mesenchymal circuit that governs profibrotic metabolism in lung fibrosis.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 40875483 · DOI · PMC12578403
Impaired AMPK control of alveolar epithelial cell metabolism promotes pulmonary fibrosis [3]
Abstract reading summary — source excerpts
- Opening excerpt
- Alveolar epithelial type II (AT2) cell dysfunction is implicated in the pathogenesis of familial and sporadic idiopathic pulmonary fibrosis (IPF).
- Closing excerpt
- We identify deficient AMPK signaling as a critical component of AT2 cell dysfunction and demonstrate that targeting this druggable signaling hub can rescue the aberrant AT2 cell metabolic phenotype and mitigate lung fibrosis in vivo.
- 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 (159 words)
Alveolar epithelial type II (AT2) cell dysfunction is implicated in the pathogenesis of familial and sporadic idiopathic pulmonary fibrosis (IPF). We previously demonstrated that expression of an AT2 cell-exclusive disease-associated protein isoform (SP-CI73T) in murine and patient-specific induced pluripotent stem cell-derived (iPSC-derived) AT2 cells leads to a block in late macroautophagy and promotes time-dependent mitochondrial impairments; however, how a metabolically dysfunctional AT2 cell results in fibrosis remains elusive. Here, using murine and human iPSC-derived AT2 cell models expressing SP-CI73T, we characterize the molecular mechanisms governing alterations in AT2 cell metabolism that lead to increased glycolysis, decreased mitochondrial biogenesis, disrupted fatty acid oxidation, accumulation of impaired mitochondria, and diminished AT2 cell progenitor capacity manifesting as reduced AT2 cell self-renewal and accumulation of transitional epithelial cells. We identify deficient AMPK signaling as a critical component of AT2 cell dysfunction and demonstrate that targeting this druggable signaling hub can rescue the aberrant AT2 cell metabolic phenotype and mitigate lung fibrosis in vivo.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 40591409 · DOI · PMC12333953
Human iPSC-based Modeling of Pulmonary Fibrosis Reveals p300/CBP Inhibition Suppresses Alveolar Transitional Cell State [4]
Abstract reading summary — source excerpts
- Opening excerpt
- Idiopathic pulmonary fibrosis (IPF) is characterized by progressive scarring of lung tissue with an urgent need for effective treatments.
- Closing excerpt
- Overall, these results elucidate the biological mechanisms of pulmonary fibrosis and provide a potential therapeutic target.
- 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 (142 words)
Idiopathic pulmonary fibrosis (IPF) is characterized by progressive scarring of lung tissue with an urgent need for effective treatments. Studies have shown that the alveolar transitional cell state (ATCS) emerges in fibrotic regions of the IPF lung. However, whether ATCS is the cause or consequence of fibrosis is controversial, and no therapeutic agents targeting the alveolar epithelial differentiation are used to treat IPF. In this study, we performed a drug screening with an in vitro pulmonary fibrosis model using fibroblast-dependent alveolar organoids derived from human induced pluripotent stem cells (iPSCs) and identified p300/CBP inhibitors as candidate therapeutic agents. Multi-omics technology revealed that ATCS induced from human iPSCs-derived alveolar organoids had a compatible profile with that reported in IPF and p300/CBP inhibitors suppressed the emergence of ATCS. Overall, these results elucidate the biological mechanisms of pulmonary fibrosis and provide a potential therapeutic target.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 41680175 · DOI · PMC12901050
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 [5]
- Research article Viral Infection Increases the Risk of Idiopathic Pulmonary Fibrosis: A Meta-Analysis [6]
- Research article Impact of Antifibrotic Therapy on Mortality and Acute Exacerbation in Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis [7]
- Research article Current and Future Treatment Landscape for Idiopathic Pulmonary Fibrosis [8]
- Research article Drug Treatment of Idiopathic Pulmonary Fibrosis: Systematic Review and Network Meta-Analysis [9]
- Research article Safety, tolerability and appropriate use of nintedanib in idiopathic pulmonary fibrosis [10]
References
- Wagner DE, Alsafadi HN, Mitash N, Justet A, Hu Q, Pineda R, et al.. Inhibition of epithelial cell YAP-TEAD/LOX signaling attenuates pulmonary fibrosis in preclinical models. . 2025. PMID 40753090 · DOI · PMC12318044
- Yadav P, Gómez Ortega J, Dabral P, Tamaki W, Chien C, Chang KC, et al.. Myeloid-mesenchymal crosstalk drives ARG1-dependent profibrotic metabolism via ornithine in lung fibrosis. . 2025. PMID 40875483 · DOI · PMC12578403
- Rodríguez LR, Alysandratos KD, Katzen J, Murthy A, Roque Barboza W, Tomer Y, et al.. Impaired AMPK control of alveolar epithelial cell metabolism promotes pulmonary fibrosis. . 2025. PMID 40591409 · DOI · PMC12333953
- Tsutsui Y, Masui A, Konishi S, Tsujimura T, Iwasaki M, Yamamoto T, et al.. Human iPSC-based Modeling of Pulmonary Fibrosis Reveals p300/CBP Inhibition Suppresses Alveolar Transitional Cell State. . 2026. PMID 41680175 · DOI · PMC12901050
- 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
- Bonella F, Spagnolo P, Ryerson C. Current and Future Treatment Landscape for Idiopathic Pulmonary Fibrosis. Drugs. 2023. PMID 37882943 · DOI · PMC10693523
- 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.