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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.
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“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
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“Idiopathic pulmonary fibrosis (IPF) is an age-related interstitial lung disease characterized by progressive scarring of lung tissue which reduces gas exchange, and leads to progressive respiratory failure (Lederer and Martinez, 2018; Raghu and Chang, 2004; Thannickal et al., 2004).”
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Behr, J., Prasse, A., Kreuter, M., Johow, J., Rabe, K. F., Bonella, F., Bonnet, R., Grohe, C., Held, M., Wilkens, H., et al. (2021) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(20)30554-3 · Cited by 522
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“Idiopathic pulmonary fibrosis (IPF) is the most common adult interstitial lung disease (ILD), a class of pulmonary diseases pathologically defined by interstitial fibrosis, inflammation, or the combination of fibrosis and inflammation (Lederer and Martinez, 2018) .”
BackgroundIdiopathic Pulmonary Fibrosis (IPF): An Overview
Barratt, S. L., Creamer, A. W., Hayton, C., & Chaudhuri, N. (2018) · Journal of Clinical Medicine · doi:10.3390/jcm7080201 · Cited by 408
“A comprehensive review of the pathogenesis of IPF is beyond the scope of this article and has been discussed recently elsewhere [28,29].”
BackgroundPeng, L., Li, W., Shi, Q.-F., Gao, F., Huang, B., Meng, J., Hu, C.-P., & Wang, C. (2020) · Cell Death and Disease · doi:10.1038/s41419-020-03178-2 · Cited by 387
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Self-citationInterleukin-11 is a therapeutic target in idiopathic pulmonary fibrosis
Ng, B., Dong, J., D’Agostino, G., Viswanathan, S., Widjaja, A. A., Lim, W.-W., Ko, N. S. J., Tan, J., Chothani, S. P., Huang, B., et al. (2019) · Science Translational Medicine · doi:10.1126/scitranslmed.aaw1237 · Cited by 314
“6A) and found that the expression of ECM-related genes was reduced in X203-treated mice as compared to IgG controls (Fig.”
BackgroundInfluentialIdiopathic Pulmonary Fibrosis and Lung Cancer: Mechanisms and Molecular Targets
Ballester, B., Milara, J., & Cortijo, J. (2019) · International Journal of Molecular Sciences · doi:10.3390/ijms20030593 · Cited by 318
“Concurrently, there is evidence that galectin 3 is a promising target for IPF [158] because it has a profibrotic action [159] that is partly mediated by binding to Muc1 [160].”
BackgroundTGF ‐β as a driver of fibrosis: physiological roles and therapeutic opportunities
Budi, E. H., Schaub, J. R., Decaris, M. L., Turner, S., & Derynck, R. (2021) · The Journal of Pathology · doi:10.1002/path.5680 · Cited by 310
“IPF, which involves fi brotic remodeling of the lung parenchyma, is characterized by severe dyspnea and cough, and is particularly devastating, with a median survival time of 3.8 years for patients of 65 years or older in the United States [15].”
BackgroundInfluentialContributions of alveolar epithelial cell quality control to pulmonary fibrosis
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Nintedanib plus Sildenafil in Patients with Idiopathic Pulmonary Fibrosis
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Modeling of fibrotic lung disease using 3D organoids derived from human pluripotent stem cells
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“2000 * * * * * * * 7 Stewart et al.(8) 1999 * * * ** * * * 8 Bando et al.”
InfluentialCNS fibroblasts form a fibrotic scar in response to immune cell infiltration.
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Xu, Z., Ren, F., Wang, P., Cao, J., Tan, C., Ma, D., Zhao, L., Dai, J., Ding, Y., Fang, H., et al. (2025) · Nature Medicine · doi:10.1038/s41591-025-03743-2 · Cited by 180
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Cui, L., Chen, S.-Y., Lerbs, T., Lee, J.-W., Domizi, P., Gordon, S., Kim, Y.-H., Nolan, G., Betancur, P., & Wernig, G. (2020) · Nature Communications · doi:10.1038/s41467-020-16466-4 · Cited by 153
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Guiot, J., Cambier, M., Boeckx, A., Henket, M. A., Nivelles, O., Gester, F., Louis, E. J., Malaise, M., Dequiedt, F., Louis, R. E., et al. (2020) · Thorax · doi:10.1136/thoraxjnl-2019-214077 · Cited by 152
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Jäger, B., Schupp, J. C., Plappert, L., Terwolbeck, O., Artysh, N., Kayser, G., Engelhard, P., Adams, T. S., Zweigerdt, R., Kempf, H., et al. (2022) · Nature Communications · doi:10.1038/s41467-022-33193-0 · Cited by 144
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Sauleda, J., Núñez, B., Sala, E., & Soriano, J. B. (2018) · Medical Sciences · doi:10.3390/medsci6040110 · Cited by 145
“It is considered a rare disease, but recent prevalence and incidence rates appear to be on the rise, probably due to greater awareness, population ageing, male sex, or other factors (see below) [2].”
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“This in turn creates fundamental changes in organ function and can even result in organ failure [100, 101] .”
BackgroundChronic WNT/β-catenin signaling induces cellular senescence in lung epithelial cells
Lehmann, M., Hu, Q., Hu, Y., Hafner, K., Costa, R., van den Berg, A., & Königshoff, M. (2020) · Cellular Signalling · doi:10.1016/j.cellsig.2020.109588 · Cited by 134
From ARDS to pulmonary fibrosis: the next phase of the COVID-19 pandemic?
Michalski, J. E., Kurche, J. S., & Schwartz, D. A. (2021) · Translational research · doi:10.1016/j.trsl.2021.09.001 · Cited by 139
“Although pulmonary fibrosis pathobiology has been focused on interactions within the alveolus, there is a growing understanding of the role of the distal airway epithelium.(55,75,76) The distal airway is known to be essential to alveolar repair after severe injury and it is hypothesized that dysregulation of this repa…”
BackgroundTargeting Oxidative Stress as a Therapeutic Approach for Idiopathic Pulmonary Fibrosis
Estornut, C., Milara, J., Bayarri, M. A., Belhadj, N., & Cortijo, J. (2022) · Frontiers in Pharmacology · doi:10.3389/fphar.2021.794997 · Cited by 138
“It is generally nonresponsive to traditional therapies such as anti-inflammatory and immunomodulatory treatment (Richeldi et al., 1941; Day, 2008; Barratt et al., 2018; Lederer and Martinez, 2018).”
BackgroundLiu, J., Wu, Z., Liu, Y., Zhan, Z., Yang, L., Wang, C., Jiang, Q., Ran, H., Li, P., & Wang, Z. (2022) · Journal of Nanobiotechnology · doi:10.1186/s12951-022-01435-4 · Cited by 134
Orbach, H., Karlinskaya, M., & Fruchter, O. (2018) · New England Journal of Medicine · doi:10.1056/nejmc1807508 · Cited by 136
Hexokinase 2 couples glycolysis with the profibrotic actions of TGF-β
Yin, X., Choudhury, M., Kang, J. H., Schaefbauer, K. J., Jung, M.-Y., Andrianifahanana, M., Hernandez, D. M., & Leof, E. B. (2019) · Science Signaling · doi:10.1126/scisignal.aax4067 · Cited by 133
“This is of particular relevance for diseases such as IPF because, although drugs such as nintedanib and pirfenidone have been approved, disease progression with poor longterm prognosis is still the norm for most patients (10, 59, 60), and attempts to identify novel therapeutics are ongoing (61, 62).”
BackgroundHome Monitoring in Patients with Idiopathic Pulmonary Fibrosis. A Randomized Controlled Trial
Moor, C. C., Mostard, R. L. M., Grutters, J. C., Bresser, P., Aerts, J. G. J. V., Chavannes, N. H., & Wijsenbeek, M. S. (2020) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202002-0328oc · Cited by 136
Air pollutants, genetic susceptibility and risk of incident idiopathic pulmonary fibrosis
Cui, F., Sun, Y., Xie, J., Li, D., Wu, M., Song, L., Hu, Y., & Tian, Y. (2022) · European Respiratory Journal · doi:10.1183/13993003.00777-2022 · Cited by 139
Dysregulation of metabolic pathways in pulmonary fibrosis
Rajesh, R., Atallah, R., & Bärnthaler, T. (2023) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2023.108436 · Cited by 132
Analysis of Generalized Fibrosis in Mouse Tissue Sections with Masson’s Trichrome Staining
van de Vlekkert, D., Machado, E., & d’Azzo, A. (2020) · BIO-PROTOCOL · doi:10.21769/bioprotoc.3629 · Cited by 134
Lee, T.-H., Yeh, C., Lee, Y.-T., Shih, Y.-C., Chen, Y.-T., Hung, C., You, M.-Y., Wu, P., Shentu, T., Huang, R., et al. (2020) · Nature Communications · doi:10.1038/s41467-020-18047-x · Cited by 131
The role of matrix metalloproteinase 9 in fibrosis diseases and its molecular mechanisms
Wang, Y., Jiao, L., Qiang, C., Chen, C., Shen, Z., Ding, F., Lv, L., Zhu, T., Lu, Y., & Cui, X. (2024) · Biomedicine & Pharmacotherapy · doi:10.1016/j.biopha.2023.116116 · Cited by 131
A lung targeted miR-29 mimic as a therapy for pulmonary fibrosis
Chioccioli, M., Roy, S., Newell, R., Pestano, L., Dickinson, B., Rigby, K., Herazo-Maya, J., Jenkins, G., Ian, S., Saini, G., et al. (2022) · eBioMedicine · doi:10.1016/j.ebiom.2022.104304 · Cited by 129
“Proc Natl Acad Sci U S A 2008;105(35):13027–32.”
Yoshida, G. J., Azuma, A., Miura, Y., & Orimo, A. (2019) · International Journal of Molecular Sciences · doi:10.3390/ijms20092256 · Cited by 131
“SPARC is exclusively expressed in IPF patients, never in healthy individuals [9,17].”
BackgroundAllen, R. J., Stockwell, A., Oldham, J. M., Guillén‐Guío, B., Schwartz, D. A., Maher, T. M., Flores, C., Noth, I., Yaspan, B. L., Jenkins, G. R., et al. (2022) · Thorax · doi:10.1136/thoraxjnl-2021-218577 · Cited by 122
Mortality and survival in idiopathic pulmonary fibrosis: a systematic review and meta-analysis
Zheng, Q., Cox, I. A., Campbell, J. A., Xia, Q., Otáhal, P., de Graaff, B., Corte, T. J., Teoh, A. K. Y., Walters, E. H., & Palmer, A. J. (2022) · ERJ Open Research · doi:10.1183/23120541.00591-2021 · Cited by 126
Exploration of radiation-induced lung injury, from mechanism to treatment: a narrative review
Yan, Y., Fu, J., Kowalchuk, R. O., Wright, C. M., Zhang, R., Li, X., & Xu, Y. (2022) · Translational Lung Cancer Research · doi:10.21037/tlcr-22-108 · Cited by 126
Idiopathic pulmonary fibrosis: Molecular mechanisms and potential treatment approaches
Glass, D. S., Grossfeld, D. J., Renna, H. A., Agarwala, P., Spiegler, P. A., Kasselman, L. J., Glass, A. D., DeLeon, J. R., & Reiss, A. B. (2020) · Respiratory Investigation · doi:10.1016/j.resinv.2020.04.002 · Cited by 132
Zhang, C., Wu, Z., Li, J., Tan, K., Yang, W., Zhao, H., & Wang, G. (2020) · Journal of Medical Virology · doi:10.1002/jmv.26634 · Cited by 122
A Biomimetic Human Lung‐on‐a‐Chip with Colorful Display of Microphysiological Breath
Zhu, Y., Sun, L., Wang, Y., Cai, L., Zhang, Z., Shang, Y., & Zhao, Y. (2022) · Advanced Materials · doi:10.1002/adma.202108972 · Cited by 121
New Insights into Hippo/YAP Signaling in Fibrotic Diseases
Mia, M. M., & Singh, M. K. (2022) · Cells · doi:10.3390/cells11132065 · Cited by 120
Han, D., Gong, H., Wei, Y., Xu, Y., Zhou, X., Wang, Z., & Feng, F. (2023) · Phytomedicine · doi:10.1016/j.phymed.2023.154680 · Cited by 118
Ahangari, F., Becker, C., Foster, D. G., Chioccioli, M., Nelson, M., Beke, K. M., Wang, X., Justet, A., Adams, T. S., Readhead, B. P., et al. (2022) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202010-3832oc · Cited by 119
Zhang, J., Lu, J. L., Xie, H., Wang, D.-P., Ni, H., Zhu, Y., Ren, L., Meng, X., & Wang, R. (2019) · Cell Death and Disease · doi:10.1038/s41419-019-1430-7 · Cited by 114
Alysandratos, K., Russo, S. J., Petcherski, A., Taddeo, E. P., Acín‐Pérez, R., Villacorta-Martín, C., Jean, J., Mulugeta, S., Rodríguez, L. R., Blum, B. C., et al. (2021) · Cell Reports · doi:10.1016/j.celrep.2021.109636 · Cited by 116
“Idiopathic pulmonary fibrosis (IPF) is the most common and severe form of idiopathic interstitial pneumonia and is characterized by relentless fibrosis leading to disruption of the gas exchange unit and death within an average of 4 years from the time of diagnosis (Lederer and Martinez, 2018; Raghu et al., 2014, 2016;…”
Ogawa, T., Shichino, S., Ueha, S., & Matsushima, K. (2021) · International Immunology · doi:10.1093/intimm/dxab040 · Cited by 113
[68 Ga]Ga-FAPI-46 PET for non-invasive detection of pulmonary fibrosis disease activity
Rosenkrans, Z. T., Massey, C. F., Bernau, K., Ferreira, C. A., Jeffery, J. J., Schulte, J. J., Moore, M. D., Valla, F., Batterton, J. M., Drake, C. R., et al. (2022) · European Journal of Nuclear Medicine and Molecular Imaging · doi:10.1007/s00259-022-05814-9 · Cited by 113
Treatment of fibrotic interstitial lung disease: current approaches and future directions
Johannson, K. A., Chaudhuri, N., Adegunsoye, A., & Wolters, P. J. (2021) · The Lancet · doi:10.1016/s0140-6736(21)01826-2 · Cited by 116
Wang, T., Duan, W., Jia, X., Huang, X., Liu, Y., Meng, F., & Ni, C. (2023) · European Respiratory Journal · doi:10.1183/13993003.01720-2023 · Cited by 117
Regulation of the NLRP3 inflammasome by autophagy and mitophagy
Gupta, S., Cassel, S. L., Sutterwala, F. S., & Dagvadorj, J. (2024) · Immunological Reviews · doi:10.1111/imr.13410 · Cited by 116
“148,149 152 Accumulation of damaged mitochondria has been associated with normal aging lung as well as in IPF.”
HDAC Inhibitors: Therapeutic Potential in Fibrosis-Associated Human Diseases
Yoon, S., Kang, G., & Eom, G. H. (2019) · International Journal of Molecular Sciences · doi:10.3390/ijms20061329 · Cited by 112
“However, there is no reported cause of IPF, and inhibition of fibrosis is the only therapeutic strategy [80,81].”
BackgroundPokharel, M. D., Flores, A. G., Marciano, D. P., Franco, M. C., Fineman, J. R., Aggarwal, S., Wang, T., & Black, S. M. (2024) · Redox Biology · doi:10.1016/j.redox.2024.103049 · Cited by 124
“It is currently hypothesized that local micro-injuries to AECs in the lungs induce AEC dysfunction, ultimately resulting in AEC senescence [195].”
BackgroundPulmonary fibrosis distal airway epithelia are dynamically and structurally dysfunctional
Stancil, I. T., Michalski, J. E., Davis‐Hall, D., Chu, H. W., Park, J., Magin, C. M., Yang, I. V., Smith, B. J., Dobrinskikh, E., & Schwartz, D. A. (2021) · Nature Communications · doi:10.1038/s41467-021-24853-8 · Cited by 113
Tanner, L., Single, A., Bhongir, R. K. V., Heusel, M., Mohanty, T., Karlsson, C. A. Q., Pan, L., Clausson, C.-M., Bergwik, J., Wang, K., et al. (2023) · Nature Communications · doi:10.1038/s41467-023-36314-5 · Cited by 111
Althobiani, M. A., Russell, A., Jacob, J., Ranjan, Y., Folarin, A., Hurst, J. R., & Porter, J. C. (2024) · Frontiers in Medicine · doi:10.3389/fmed.2024.1296890 · Cited by 127
Parimon, T., Yao, C., Habiel, D. M., Ge, L., Bora, S. A., Brauer, R., Evans, C. M., Xie, T., Alonso-Valenteen, F., Medina‐Kauwe, L. K., et al. (2019) · JCI Insight · doi:10.1172/jci.insight.129359 · Cited by 108
“In particular, targeting TGF-β and Wnt has been elusive, and both pirfenidone and nintedanib, which are approved for IPF, have pleiotropic targets (2).”
Background
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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