The DOI matches its Crossref record; 141 of the 143 references it cites were found in a registry or an index.
Idiopathic pulmonary fibrosis
Abstract
Idiopathic pulmonary fibrosis is a prototype of chronic, progressive, and fibrotic lung disease. Healthy tissue is replaced by altered extracellular matrix and alveolar architecture is destroyed, which leads to decreased lung compliance, disrupted gas exchange, and ultimately respiratory failure and death. In less than a decade, understanding of the pathogenesis and management of this disease has been transformed, and two disease-modifying therapies have been approved, worldwide. In this Seminar, we summarise the presentation, pathophysiology, diagnosis, and treatment options available for patients with idiopathic pulmonary fibrosis. This disease has improved understanding of the mechanisms of lung fibrosis, and offers hope that similar approaches will transform the management of patients with other progressive fibrotic lung diseases.
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Each is checked against this paper’s own text: does it say what the citing paper says it does?
In the citing paper
“marked by interstitial pneumonia, dyspnea and cough25, histologically characterized by fibroblast proliferation and extracellular matrix remodeling26–29.”
From A generative AI-discovered TNIK inhibitor for idiopathic pulmonary fibrosis: a randomized phase 2a trial (2025) · cites this paper as [26]
In the citing paper
“The median survival time after diagnosis is 2–4 years despite widespread availability of targeted antifibrotic therapies26,30, highlighting the ongoing need for novel, effective therapies to treat IPF.”
From A generative AI-discovered TNIK inhibitor for idiopathic pulmonary fibrosis: a randomized phase 2a trial (2025) · cites this paper as [26]
In the citing paper
“Clinical trials investigating these two drugs in patients with IPF have demonstrated only a slowing of disease progression33,34, as measured by forced vital capacity (FVC) and self-reported quality of life (QOL) metrics, and unclear benefit to survival26, making the development of therapies that can restore lung function and reverse the course of disease a priority.”
From A generative AI-discovered TNIK inhibitor for idiopathic pulmonary fibrosis: a randomized phase 2a trial (2025) · cites this paper as [26]
In the citing paper
“IPF is an age-related, progressive lung disease diagnosed by excluding other potential causes of interstitial lung disease (for example, environmental exposures, connective tissue disease, hypersensitivity pneumonitis or drug toxicities)24 and marked by interstitial pneumonia, dyspnea and cough25, histologically characterized by fibroblast proliferation and extracellular matrix remodeling26–29.”
From A generative AI-discovered TNIK inhibitor for idiopathic pulmonary fibrosis: a randomized phase 2a trial (2025) · cites this paper as [26]
In the citing paper
“These observations indicate that the Pdgf pathway is crucial to disease pathogenesis.”
From Reference-based analysis of lung single-cell sequencing reveals a transitional profibrotic macrophage (2019) · cites this paper as [39]
Citations
Cites
Cancer Incidence in Five Continents
Doll, R., Muir, C. S., & Waterhouse, J. A. H. (1970) · Springer eBooks · doi:10.1007/978-3-642-85851-2 · Cited by 8,485
Raghu, G., Collard, H. R., Egan, J. J., Martinez, F. J., Behr, J., Brown, K. K., Colby, T. V., Cordier, J.-F., Flaherty, K. R., Lasky, J. A., et al. (2011) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.2009-040gl · Cited by 7,186
Self-citationEfficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis
Richeldi, L., du Bois, R. M., Raghu, G., Azuma, A., Brown, K. K., Costabel, U., Cottin, V., Flaherty, K. R., Hansell, D. M., Inoue, Y., et al. (2014) · New England Journal of Medicine · doi:10.1056/nejmoa1402584 · Cited by 4,654
Self-citationTravis, W. D., Costabel, U., Hansell, D. M., King, T. E., Lynch, D. A., Nicholson, A. G., Ryerson, C. J., Ryu, J. H., Selman, M., Wells, A. U., et al. (2013) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201308-1483st · Cited by 4,378
Self-citation(2002) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/ajrccm.165.2.ats01 · Cited by 4,196
Fleischner Society: Glossary of Terms for Thoracic Imaging
Hansell, D. M., Bankier, A. A., MacMahon, H., McLoud, T. C., Müller, N. L., & Rémy, J. (2008) · Radiology · doi:10.1148/radiol.2462070712 · Cited by 4,189
A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis.
King, T. E., Bradford, W. Z., Castro-Bernardini, S., Fagan, E. A., Glaspole, I., Glassberg, M. K., Gorina, E., Hopkins, P. M., Kardatzke, D., Lancaster, L., et al. (2014) · New England Journal of Medicine · doi:10.1056/nejmoa1402582 · Cited by 3,975
Idiopathic Pulmonary Fibrosis: Diagnosis and Treatment: International Consensus Statement
(2000) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/ajrccm.161.2.ats3-00 · Cited by 2,651
Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials
Noble, P. W., Albera, C., Bradford, W. Z., Costabel, U., Glassberg, M. K., Kardatzke, D., King, T. E., Lancaster, L., Sahn, S. A., Szwarcberg, J., et al. (2011) · The Lancet · doi:10.1016/s0140-6736(11)60405-4 · Cited by 2,154
Raghu, G., Rochwerg, B., Zhang, Y., Garcia, C. A. C., Azuma, A., Behr, J., Brozek, J. L., Collard, H. R., Cunningham, W., Homma, S., et al. (2015) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201506-1063st · Cited by 1,916
Self-citation
Research works printed but not linked: 1
- 2000, Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS), Am J Respir Crit Care Med, 161, 646, 10.1164/ajrccm.161.2.ats3-00 · DOI
Cited by
Sorted by citations
Items 1,001–1,003 of 1,003
Li, Z., Zhang, Q., Xiang, J., Zhao, M., Meng, Y., Hu, X., Li, T., Nie, Y., Sun, H., Yan, T., et al. (2023) · Materials Today Bio · doi:10.1016/j.mtbio.2023.100653 · Cited by 8
Bibaki, E., Tsitoura, E., Vasarmidi, E., Margaritopoulos, G., Trachalaki, A., Koutoulaki, C., Georgopoulou, T., Spandidos, D., Tzanakis, N., & Antoniou, K. (2018) · Molecular Medicine Reports · doi:10.3892/mmr.2018.8778 · Cited by 29
Zhang, X.-L., Cao, Y., & Zheng, B. (2023) · BMC Pulmonary Medicine · doi:10.1186/s12890-023-02778-w · Cited by 7
Metadata
- Authors
- Richeldi, L., Collard, H. R., & Jones, M. G.
- Affiliations
- Università Cattolica del Sacro Cuore (IT)
- University of California, San Francisco (US)
- National Institute for Health and Care Research (GB)
- Corresponding author
- Richeldi, Luca
- Venue
- The Lancet
- Publisher
- Elsevier BV
- Published
- 2017-05
- Volume
- 389
- Issue
- 10082
- Pages
- 1941-1952
- ISSN
- 0099-5355 (ISSN-L)
- 0140-6736
- OpenAlex
- W2599075603
- Cited by
- 2,020
- References
- 143
- Citations by year
- 2026: 202 · 2025: 317 · 2024: 338 · 2023: 293 · 2022: 273 · 2021: 233 · 2020: 167 · 2019: 94 · 2018: 76 · 2017: 22
- FWCI
- 17.97
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Lancet (publishedVersion)
- PubMed (publishedVersion)
- ePrints Soton (University of Southampton) (acceptedVersion)
- Funders
- National Institute for Health and Care Research (CL-2016-26-004)
- 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 › Pulmonary Hypertension Research and Treatments
- Keywords
- idiopathic pulmonary fibrosis, pulmonary fibrosis, pathophysiology, extracellular matrix, alveolar architecture, disease-modifying therapy, diagnosis, treatment options, respiratory failure
- MeSH terms
- Humans, Idiopathic Pulmonary Fibrosis
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