Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic?
Open accessAbstract
Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible, and typically fatal lung disease characterised by subpleural fibrosis, subepithelial fibroblast foci, and microscopic honeycombing. Although understanding of the pathogenic mechanisms continues to evolve, evidence indicates that distal airway and alveolar epithelial cells are central drivers of the disease. In this Viewpoint, we review the history of naming and classifications used to define the disease now referred to as IPF, in the context of understanding the clinical presentation, causes, and pathogenesis of the disease. We aim to generate discussion on whether, given the substantial progress made in understanding the clinical, genetic, cellular, and molecular mechanisms involved in the development of IPF, a change of name should be considered. To initiate this discussion, we offer new suggestions to update the name of this disease and new approaches to classify all forms of pulmonary fibrosis.
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BLM-injury promotes the synthesis and release of TGF-β1 by lung epithelial cells.
“Based on the previous report that BLM-injury promotes the synthesis and release of TGF-β1 by lung epithelial cells 69”
From Nicotinamide phosphoribosyltransferase prompts bleomycin-induced pulmonary fibrosis by driving macrophage M2 polarization in mice (2024) · cites this paper as [69]
The section “Primary role of lung epithelia in disease pathogenesis” says abnormal lung epithelium expresses mediators that include TGF-β. It does not say that bleomycin (BLM) injury promotes epithelial synthesis and release of TGF-β1; searches for bleomycin and that mechanism found no supporting passage. Page numbers were not available in our copy. (LLM, conf=0.84)
Experimental — accuracy not yet measuredEnvironmental factors linked to IPF pathogenesis include cigarette smoke, occupational exposure to wood, metal and silica dust, air pollution (particulate matter and nitrogen dioxide), GERD, and viral infections.
“Environmental factors linked to IPF pathogenesis include cigarette smoke, occupational exposure to certain substances in the workplace, such as wood, metal and silica dust, air pollution, particularly particulate matter and nitrogen dioxide, gastroesophageal reflux disease (GERD) and viral infections (Raghu & Meyer, 2012; Wolters et al., 2018).”
From Pulmonary fibrosis: Emerging diagnostic and therapeutic strategies (2023) · cites this paper as Wolters et al., 2018
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A Novel Dyskerin (DKC1) Mutation Is Associated With Familial Interstitial Pneumonia
Kropski, J. A., Mitchell, D. B., Markin, C. R., Polosukhin, V. V., Choi, L., Johnson, J. E., Lawson, W. E., Phillips, J. A., Cogan, J. D., Blackwell, T. S., et al. (2014) · CHEST Journal · doi:10.1378/chest.13-2224 · Cited by 151 · see citing works
Self-citationDisayabutr, S., Kim, E. K., Cha, S.-I., Green, G., Naikawadi, R. P., Jones, K. D., Golden, J. A., Schroeder, A., Matthay, M. A., Kukreja, J., et al. (2016) · PLOS ONE · doi:10.1371/journal.pone.0158367 · Cited by 136 · see citing works
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Self-citationAge-driven developmental drift in the pathogenesis of idiopathic pulmonary fibrosis
Selman, M., López-Otín, C., & Pardo, A. (2016) · European Respiratory Journal · doi:10.1183/13993003.00398-2016 · Cited by 122 · see citing works
Self-citationA large kindred of pulmonary fibrosis associated with a novel ABCA3 gene variant
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Occupational and Environmental Factors and Idiopathic Pulmonary Fibrosis in Japan
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MUC 5 B promoter polymorphism in J apanese patients with idiopathic pulmonary fibrosis
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Spontaneous Chitin Accumulation in Airways and Age-Related Fibrotic Lung Disease
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Self-citationThree-dimensional characterization of fibroblast foci in idiopathic pulmonary fibrosis
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Self-citationAssociation between MUC5B and TERT polymorphisms and different interstitial lung disease phenotypes
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Idiopathic Pulmonary Fibrosis; Its Occurrence in Identical Twin Sisters
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Matrix, Mesenchyme, and Mechanotransduction
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Lung mast cell density defines a subpopulation of patients with idiopathic pulmonary fibrosis
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Self-citationAccumulation of BDCA1+ Dendritic Cells in Interstitial Fibrotic Lung Diseases and Th2-High Asthma
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Self-citationIdiopathic Pulmonary Fibrosis: Roentgenologic Findinqs
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Effect of “Pink Eye” Label on Parents’ Intent to Use Antibiotics and Perceived Contagiousness
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Reyfman, P. A., Walter, J. M., Joshi, N., Anekalla, K. R., McQuattie-Pimentel, A. C., Chiu, S., Fernandez, R., Akbarpour, M., Chen, C.-I., Ren, Z., et al. (2019) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201712-2410oc · Cited by 1,203 · see citing works
Spectrum of fibrotic lung diseases
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Molecular programs of fibrotic change in aging human lung
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Self-citationChen, Y., Wang, T., Liang, F., Han, J., Lou, Z., Yu, Y., Li, J., Zhan, T., Gu, Y., Dong, L., et al. (2024) · Theranostics · doi:10.7150/thno.94482 · Cited by 56 · see citing works
“Based on the previous report that BLM-injury promotes the synthesis and release of TGF-β1 by lung epithelial cells [69], as well as our in vitro results, it shows that the released TGF-β1 induces macrophages to synthesize and release NAMPT, and that these autocrine and paracrine eNAMPT further…”
BackgroundPulmonary fibrosis: Emerging diagnostic and therapeutic strategies
Selvarajah, B., Platé, M., & Chambers, R. C. (2023) · Molecular Aspects of Medicine · doi:10.1016/j.mam.2023.101227 · Cited by 57 · see citing works
Guo, H., Sun, J., Zhang, S., Nie, Y., Zhou, S., & Zeng, Y. (2023) · Frontiers in Pharmacology · doi:10.3389/fphar.2023.1205948 · Cited by 41 · see citing works
Park, M. Y., Bae, S., Heo, J. A., Park, M., Kim, Y., Han, J., Jang, I., Yu, K., & Oh, J. (2023) · Clinical and Translational Science · doi:10.1111/cts.13518 · Cited by 15 · see citing works
Nishioka, Y., Homma, S., Ogura, T., Sato, S., Arai, N., Tomii, K., Kamio, K., Sakamoto, S., Miyazaki, Y., Tomioka, H., et al. (2023) · Respiratory Investigation · doi:10.1016/j.resinv.2023.04.008 · Cited by 12 · see citing works
- Authors
- Wolters, P. J., Blackwell, T. S., Eickelberg, O., Loyd, J. E., Kaminski, N., Jenkins, G., Maher, T. M., Molina-Molina, M., Noble, P. W., Raghu, G., Richeldi, L., Schwarz, M. I., Selman, M., Wuyts, W. A., & Schwartz, D. A.
- Affiliations
- University of California, San Francisco (US)
- Vanderbilt University (US)
- University of Colorado Anschutz (US)
- Yale University (US)
- University of Nottingham (GB)
- Imperial College London (GB)
- Cedars-Sinai Medical Center (US)
- University of Washington (US)
- Università Cattolica del Sacro Cuore (IT)
- Universitair Ziekenhuis Leuven (BE)
- Corresponding author
- Wolters, Paul J
- ORCID
- Venue
- The Lancet Respiratory Medicine
- Publisher
- Elsevier BV
- Published
- 2018-02
- Volume
- 6
- Issue
- 2
- Pages
- 154-160
- ISSN
- 2213-2600 (ISSN-L)
- PMC
- PMC5903445
- OpenAlex
- W2784709509
- Cited by
- 196
- References
- 92
- Citations by year
- 2026: 13 · 2025: 24 · 2024: 19 · 2023: 32 · 2022: 22 · 2021: 25 · 2020: 23 · 2019: 22 · 2018: 16
- FWCI
- 3.83
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- The Lancet Respiratory Medicine (acceptedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- eScholarship (California Digital Library) (submittedVersion)
- Spiral (Imperial College London) (submittedVersion, cc-by-nc-nd)
- Funders
- U.S. Department of Veterans Affairs (CDA13-017)
- Gilead Sciences
- FibroGen
- Veracyte
- Shionogi
- National Heart, Lung, and Blood Institute (R01HL097163, UH3HL123442, U01HL112707, U01HL108642, R33HL120770, P01HL092870, R01HL127349)
- National Human Genome Research Institute (U54HG008540)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Occupational and environmental lung diseases
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Medical Imaging and Pathology Studies
- Keywords
- idiopathic pulmonary fibrosis, disease nomenclature, pathogenesis, alveolar epithelial cells, distal airways, honeycombing, subpleural fibrosis, genetic mechanisms, clinical features
- MeSH terms
- Fibroblasts, Humans, Lung, Risk Factors, Idiopathic Pulmonary Fibrosis
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