The DOI matches its Crossref record; 98 of the 98 references it cites were found in a registry or an index.
Idiopathic Pulmonary Fibrosis
Abstract
Idiopathic pulmonary fibrosis appears to be increasing in incidence. It requires early recognition and intervention with supportive care and pharmacologic agents to forestall its progression. Lung transplantation may be curative.
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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 [25]
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 [25]
In the citing paper
“Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal, age-associated lung disease with poor survival1.”
From Inhaled LTI-03 for idiopathic pulmonary fibrosis: a randomized dose escalation study (2026) · cites this paper as [1]
In the citing paper
“IPF is a process that is characterized by epithelial cell injury, fibroblast expansion, augmented ECM remodeling, and lung architecture destruction (87, 141).”
From Aging and Lung Disease (2020) · cites this paper as [141]
Citations
Cites
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
Efficacy 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
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
Self-citationScreening for Lung Cancer: U.S. Preventive Services Task Force Recommendation Statement
Moyer, V. A., & on behalf of the U.S. Preventive Services Task Force* (2013) · Annals of Internal Medicine · doi:10.7326/m13-2771 · Cited by 2,642
MacMahon, H., Naidich, D. P., Goo, J. M., Lee, K. S., Leung, A. N. C., Mayo, J. R., Mehta, A. C., Ohno, Y., Powell, C. A., Prokop, M., et al. (2017) · Radiology · doi:10.1148/radiol.2017161659 · Cited by 2,491
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
Prednisone, Azathioprine, and N -Acetylcysteine for Pulmonary Fibrosis
The Idiopathic Pulmonary Fibrosis Clinical Research Network (2012) · New England Journal of Medicine · doi:10.1056/nejmoa1113354 · Cited by 1,654
Acute Exacerbation of Idiopathic Pulmonary Fibrosis. An International Working Group Report
Collard, H. R., Ryerson, C. J., Corte, T. J., Jenkins, G., Kondoh, Y., Lederer, D. J., Lee, J. S., Maher, T. M., Wells, A. U., Antoniou, K. M., et al. (2016) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201604-0801ci · Cited by 1,444
Self-citationTelomerase Mutations in Families with Idiopathic Pulmonary Fibrosis
Armanios, M. Y., Chen, J. J.-L., Cogan, J. D., Alder, J. K., Ingersoll, R. G., Markin, C., Lawson, W. E., Xie, M., Vulto, I., Phillips, J. A., et al. (2007) · New England Journal of Medicine · doi:10.1056/nejmoa066157 · Cited by 1,357
Weill, D., Benden, C., Corris, P. A., Dark, J. H., Davis, R. D., Keshavjee, S., Lederer, D. J., Mulligan, M. J., Patterson, G. A., Singer, L. G., et al. (2015) · The Journal of Heart and Lung Transplantation · doi:10.1016/j.healun.2014.06.014 · Cited by 1,329
Self-citation
Research works printed but not linked: 2
- Philp CJ, Siebeke I, Clements D, et al. ECM crosslinking enhances fibroblast growth and protects against matrix proteolysis in lung fibrosis. Am J Respir Cell Mol Biol 2017 October 20 (Epub ahead of print).29053339
- Parker J, Sawtell R, Gagnon L, et al. PBI-4050 is safe and well tolerated and shows evidence of benefit in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2017;195:A7606-A7606. abstract.
Cited by
Sorted by citations
Nintedanib in Progressive Fibrosing Interstitial Lung Diseases
Flaherty, K. R., Wells, A. U., Cottin, V., Devaraj, A., Walsh, S. L. F., Inoue, Y., Richeldi, L., Kolb, M., Tetzlaff, K., Stowasser, S., et al. (2019) · New England Journal of Medicine · doi:10.1056/nejmoa1908681 · Cited by 2,403
Adams, T. S., Schupp, J. C., Poli, S., Ayaub, E. A., Neumark, N., Ahangari, F., Chu, S. G., Raby, B. A., DeIuliis, G., Januszyk, M., et al. (2020) · Science Advances · doi:10.1126/sciadv.aba1983 · Cited by 1,420
“IPF is still best described by its histopathological pattern of usual interstitial pneumonia (UIP), which includes the presence of fibroblast foci, hyperplastic alveolar epithelial cells that localize adjacent to fibroblastic foci, and a distortion of airway architecture combined with an accumulation of microscopic ai…”
BackgroundInfluentialReyfman, 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
“Pulmonary fibrosis is defined as the progressive replacement of alveolar tissue with fibrotic scar that threatens alveolar gas exchange and reduces lung compliance (1).”
BackgroundNew insights into TGF-β/Smad signaling in tissue fibrosis
Hu, H.-H., Chen, D., Wang, Y., Feng, Y., Cao, G., Vaziri, N. D., & Zhao, Y. (2018) · Chemico-Biological Interactions · doi:10.1016/j.cbi.2018.07.008 · Cited by 1,205
Habermann, A. C., Gutierrez, A. J., Bui, L. T., Yahn, S. L., Winters, N. I., Calvi, C. L., Peter, L., Chung, M.-I., Taylor, C. J., Jetter, C., et al. (2020) · Science Advances · doi:10.1126/sciadv.aba1972 · Cited by 1,129
“The most severe form of PF, idiopathic PF (IPF), is a relentlessly progressive disorder of unknown cause that leads to respiratory failure and death or lung transplantation within 3 to 5 years of diagnosis (2).”
Metadata
- Authors
- Lederer, D. J., & Martinez, F. J.
- Affiliations
- Columbia University Irving Medical Center (US)
- From the Departments of Medicine and Epidemiology, Columbia University Irving Medical Center (D.J.L.), and the Department of Medicine, Weill Cornell Medical Center (F.J.M.) — both in New York.
- ORCID
- Lederer, David J.
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 2018-05-10
- Volume
- 378
- Issue
- 19
- Pages
- 1811-1823
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- OpenAlex
- W2799479598
- Cited by
- 2,056
- References
- 98
- Citations by year
- 2026: 248 · 2025: 267 · 2024: 299 · 2023: 308 · 2022: 301 · 2021: 266 · 2020: 197 · 2019: 129 · 2018: 40
- FWCI
- 28.43
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- New England Journal of Medicine (publishedVersion)
- PubMed (publishedVersion)
- 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 › Surgery › Transplantation: Methods and Outcomes
- Keywords
- idiopathic pulmonary fibrosis, incidence, early recognition, supportive care, pharmacotherapy, disease progression, lung transplantation
- MeSH terms
- Diagnosis, Differential, Dyspnea, Enzyme Inhibitors, Humans, Indoles, Lung, Oxygen Inhalation Therapy, Prognosis, Pyridones, Risk Factors, Tomography, X-Ray Computed, Transforming Growth Factor beta, Idiopathic Pulmonary Fibrosis
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