Cited by 2,020Interstitial Lung Diseases and Idiopathic Pulmonary FibrosisOccupational and environmental lung diseasesPulmonary Hypertension Research and Treatments

The DOI matches its Crossref record; 141 of the 143 references it cites were found in a registry or an index.

Idiopathic pulmonary fibrosis

Richeldi, Luca, Collard, Harold R, Jones, Mark G·2017·The Lancet

DOI 10.1016/s0140-6736(17)30866-8

Open this paper in CoChat — free account

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.

Integrity signals

Registry

Crossref · 10.1016/s0140-6736(17)30866-8

Cites

141 of 141 references verified0 citation errors · 0 not checkable

Statistics

Recomputing p-values needs the paper’s full text.

Claims the paper makes

This paper’s full text isn’t openly available, so its claims and self-check weren’t run. The registry and citation checks still apply.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

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
An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management
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-citation
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
Self-citation
An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias
Travis, 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
American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias
(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
An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis: An Update of the 2011 Clinical Practice Guideline
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

Show all 143 references

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

Sorted by citations

Items 201–300 of 1,003

Telomerase treatment prevents lung profibrotic pathologies associated with physiological aging
Piñeiro-Hermida, S., Autilio, C., Martínez, P., Bosch, F., Pérez-Gil, J., & Blasco, M. A. (2020) · Journal of Cell Biology · doi:10.1083/jcb.202002120 · Cited by 60
What Patients With Idiopathic Pulmonary Fibrosis and Caregivers Want: Filling the Gaps With Patient Reported Outcomes and Experience Measures
Kalluri, M., Luppi, F., & Ferrara, G. (2019) · The American Journal of Medicine · doi:10.1016/j.amjmed.2019.08.032 · Cited by 61
The arachidonic acid metabolite 11,12-epoxyeicosatrienoic acid alleviates pulmonary fibrosis
Kim, H. S., Moon, S.-J., Lee, S. E., Hwang, G. W., Yoo, H. J., & Song, J. W. (2021) · Experimental & Molecular Medicine · doi:10.1038/s12276-021-00618-7 · Cited by 60
Eucalyptol prevents bleomycin-induced pulmonary fibrosis and M2 macrophage polarization
Rui, Y., Han, X., Jiang, A., Hu, J., Li, M., Liu, B., Qian, F., & Huang, L. (2022) · European Journal of Pharmacology · doi:10.1016/j.ejphar.2022.175184 · Cited by 61
Pathological collagen targeting and penetrating liposomes for idiopathic pulmonary fibrosis therapy
Yang, M., Lin, Y.-J., Han, M., Bi, Y., He, X.-Y., Xing, L., Jeong, J., Zhou, T., & Jiang, H. (2022) · Journal of Controlled Release · doi:10.1016/j.jconrel.2022.09.054 · Cited by 63
A Novel CD206 Targeting Peptide Inhibits Bleomycin-Induced Pulmonary Fibrosis in Mice
Ghebremedhin, A., Salam, A. B., Adu-Addai, B., Noonan, S., Stratton, R. J., Ahmed, M. S. U., Khantwal, C. M., Martin, G. R., Lin, H., Andrews, C., et al. (2023) · Cells · doi:10.3390/cells12091254 · Cited by 65
SPP1 induces idiopathic pulmonary fibrosis and NSCLC progression via the PI3K/Akt/mTOR pathway
Yue, B., Xiong, D., Chen, J., Yang, X., Zhao, J., Shao, J., Wei, D., Gao, F., Huang, M., & Chen, J. (2024) · Respiratory Research · doi:10.1186/s12931-024-02989-7 · Cited by 61
Therapeutic Targeting of the Interleukin-4/Interleukin-13 Signaling Pathway: In Allergy and Beyond
Karo‐Atar, D., Bitton, A., Benhar, I., & Munitz, A. (2018) · BioDrugs · doi:10.1007/s40259-018-0280-7 · Cited by 63
Drug-related problems in patients with rheumatoid arthritis
Ma, S. N., Huri, H. Z., & Yahya, F. (2019) · Therapeutics and Clinical Risk Management · doi:10.2147/tcrm.s194921 · Cited by 64
Blockade of platelet-derived growth factor receptor-β, not receptor-α ameliorates bleomycin-induced pulmonary fibrosis in mice
Kishi, M., Aono, Y., Sato, S., Koyama, K., Azuma, M., Abe, S., Kawano, H., Kishi, J., Toyoda, Y., Okazaki, H., et al. (2018) · PLoS ONE · doi:10.1371/journal.pone.0209786 · Cited by 65
Endothelial cell-derived MMP19 promotes pulmonary fibrosis by inducing E(nd)MT and monocyte infiltration
Zhao, W., Wang, L., Yang, J., Chen, X., Guo, X., Xu, K., Wang, N., Zhao, W., Xia, C., Lian, H., et al. (2023) · Cell Communication and Signaling · doi:10.1186/s12964-023-01040-4 · Cited by 63
Effects of β-Sitosterol from Corn Silk on TGF-β1-Induced Epithelial–Mesenchymal Transition in Lung Alveolar Epithelial Cells
Park, Y. J., Bang, I. J., Jeong, M. H., Kim, H. R., Lee, D. E., Kwak, J. H., & Chung, K. H. (2019) · Journal of Agricultural and Food Chemistry · doi:10.1021/acs.jafc.9b02730 · Cited by 61
Accumulation of damaged mitochondria in alveolar macrophages with reduced OXPHOS related gene expression in IPF
Tsitoura, E., Vasarmidi, E., Bibaki, E., Trachalaki, A., Koutoulaki, C., Papastratigakis, G., Papadogiorgaki, S., Chalepakis, G., Tzanakis, N., & Antoniou, K. M. (2019) · Respiratory Research · doi:10.1186/s12931-019-1196-6 · Cited by 63
Cell-based therapy for idiopathic pulmonary fibrosis
Lu, Q., & El-Hashash, A. H. K. (2019) · Stem Cell Investigation · doi:10.21037/sci.2019.06.09 · Cited by 59
Targeting monocytes/macrophages in fibrosis and cancer diseases: Therapeutic approaches
Conte, E. (2021) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2021.108031 · Cited by 61
Molecular Insight into the Therapeutic Effects of Stem Cell-Derived Exosomes in Respiratory Diseases and the Potential for Pulmonary Delivery
Azhdari, M. H., Goodarzi, N., Doroudian, M., & MacLoughlin, R. J. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms23116273 · Cited by 60
Neutrophil lymphocyte ratio as an indicator for disease progression in Idiopathic Pulmonary Fibrosis
Achaiah, A., Rathnapala, A., Pereira, A. Z., Bothwell, H., Dwivedi, K., Barker, R., Iotchkova, V., Benamore, R. E., Hoyles, R. K., & Ho, L. (2022) · BMJ Open Respiratory Research · doi:10.1136/bmjresp-2022-001202 · Cited by 57
Glycolysis Reprogramming in Idiopathic Pulmonary Fibrosis: Unveiling the Mystery of Lactate in the Lung
Yan, P., Liu, J., Li, Z., Wang, J., Zhu, Z., Wang, L., & Yu, G. (2023) · International Journal of Molecular Sciences · doi:10.3390/ijms25010315 · Cited by 68
miR-200 family members reduce senescence and restore idiopathic pulmonary fibrosis type II alveolar epithelial cell transdifferentiation
Moimas, S., Salton, F., Kośmider, B., Ring, N., Volpe, M. C., Bahmed, K., Braga, L., Rehman, M., Vodret, S., Graziani, M. L., et al. (2019) · ERJ Open Research · doi:10.1183/23120541.00138-2019 · Cited by 62
Idiopathische Lungenfibrose – Epidemiologie, Ursachen und klinischer Verlauf
Schäfer, S. C., Funke-Chambour, M., & Berezowska, S. (2020) · Der Pathologe · doi:10.1007/s00292-019-00747-x · Cited by 58
Endothelial to Mesenchymal Transition in Pulmonary Vascular Diseases
Yun, E., Kook, Y., Yoo, K. H., Kim, K. I., Lee, M., Kim, J., & Lee, A. (2020) · Biomedicines · doi:10.3390/biomedicines8120639 · Cited by 61
Adenosine at the Interphase of Hypoxia and Inflammation in Lung Injury
Li, X., Berg, N. K., Mills, T., Zhang, K., Eltzschig, H. K., & Yuan, X. (2021) · Frontiers in Immunology · doi:10.3389/fimmu.2020.604944 · Cited by 59
Inhibition of DNA methylation de‐represses peroxisome proliferator‐activated receptor‐γ and attenuates pulmonary fibrosis
Wei, A., Gao, Q., Chen, F., Zhu, X., Chen, X., Zhang, L., Su, X., Dai, J., Shi, Y. P., & Cao, W. (2021) · British Journal of Pharmacology · doi:10.1111/bph.15655 · Cited by 58
Exosomes in chronic respiratory diseases
Purghè, B., Manfredi, M., Ragnoli, B., Baldanzi, G., & Malerba, M. (2021) · Biomedicine & Pharmacotherapy · doi:10.1016/j.biopha.2021.112270 · Cited by 59
Inhaled mRNA Nanoformulation with Biogenic Ribosomal Protein Reverses Established Pulmonary Fibrosis in a Bleomycin‐Induced Murine Model
Zhang, R., Jing, W., Chen, C., Zhang, S., Abdalla, M., Sun, P., Wang, G., You, W., Yang, Z., Zhang, J., et al. (2022) · Advanced Materials · doi:10.1002/adma.202107506 · Cited by 58
TREM2 Insufficiency Protects against Pulmonary Fibrosis by Inhibiting M2 Macrophage Polarization
Luo, Q., Deng, D., Li, Y., Shi, H., Zhao, J., Qian, Q., Wang, W., Cai, J., Yu, W., & Liu, J. (2023) · International Immunopharmacology · doi:10.1016/j.intimp.2023.110070 · Cited by 64
A comprehensive review of tanshinone IIA and its derivatives in fibrosis treatment
Bi, Z., Wang, Y., & Zhang, W. (2021) · Biomedicine & Pharmacotherapy · doi:10.1016/j.biopha.2021.111404 · Cited by 57
The long non-coding RNA PFI protects against pulmonary fibrosis by interacting with splicing regulator SRSF1
Sun, J., Jin, T., Su, W., Guo, Y., Niu, Z., Guo, J., Li, L., Wang, J., Ma, L., Yu, T., et al. (2021) · Cell Death and Differentiation · doi:10.1038/s41418-021-00792-1 · Cited by 57
STING Signaling and Sterile Inflammation
Couillin, I., & Riteau, N. (2021) · Frontiers in Immunology · doi:10.3389/fimmu.2021.753789 · Cited by 60
Diabetic Pneumopathy–A New Diabetes-Associated Complication: Mechanisms, Consequences and Treatment Considerations
Kopf, S., Kumar, V., Κender, Ζ., Han, Z., Fleming, T., Herzig, S., & Nawroth, P. P. (2021) · Frontiers in Endocrinology · doi:10.3389/fendo.2021.765201 · Cited by 60
FoxM1-dependent RAD51 and BRCA2 signaling protects idiopathic pulmonary fibrosis fibroblasts from radiation-induced cell death
Im, J., Lawrence, J., Seelig, D. M., & Nho, R. S. (2018) · Cell Death and Disease · doi:10.1038/s41419-018-0652-4 · Cited by 59
Nicotinamide phosphoribosyltransferase prompts bleomycin-induced pulmonary fibrosis by driving macrophage M2 polarization in mice
Chen, 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 60
Alveolar Macrophages: Adaptation to Their Anatomic Niche during and after Inflammation
Martin, F. P., Jacqueline, C., Poschmann, J., & Roquilly, A. (2021) · Cells · doi:10.3390/cells10102720 · Cited by 57
Idiopathic pulmonary fibrosis is associated with tight junction protein alterations
Zou, J., Li, Y., Yu, J., Dong, L., Husain, A. N., Shen, L., & Weber, C. R. (2020) · Biochimica et Biophysica Acta (BBA) - Biomembranes · doi:10.1016/j.bbamem.2020.183205 · Cited by 56
Phase 2 trial design of BMS-986278, a lysophosphatidic acid receptor 1 (LPA1) antagonist, in patients with idiopathic pulmonary fibrosis (IPF) or progressive fibrotic interstitial lung disease (PF-ILD)
Corte, T. J., Lancaster, L., Swigris, J. J., Maher, T. M., Goldin, J. G., Palmer, S. M., Suda, T., Ogura, T., Minnich, A., Zhan, X., et al. (2021) · BMJ Open Respiratory Research · doi:10.1136/bmjresp-2021-001026 · Cited by 59
LncRNA Hoxaas3 promotes lung fibroblast activation and fibrosis by targeting miR-450b-5p to regulate Runx1
Lin, S., Zhang, R., Xu, L., Ma, R., Xu, L., Zhu, L., Hu, J., & An, X. (2020) · Cell Death and Disease · doi:10.1038/s41419-020-02889-w · Cited by 56
Fisetin Alleviated Bleomycin-Induced Pulmonary Fibrosis Partly by Rescuing Alveolar Epithelial Cells From Senescence
Zhang, L., Tong, X., Huang, J., Wu, M., Zhang, S., Wang, D., Liu, S., & Fan, H. S. (2020) · Frontiers in Pharmacology · doi:10.3389/fphar.2020.553690 · Cited by 56
Hermansky–Pudlak syndrome pulmonary fibrosis: a rare inherited interstitial lung disease
Yokoyama, T., & Gochuico, B. R. (2021) · European Respiratory Review · doi:10.1183/16000617.0193-2020 · Cited by 56
Extracellular Vesicle/Macrophage Axis: Potential Targets for Inflammatory Disease Intervention
Tang, D., Cao, F., Yan, C., Fang, K., Ma, J., Gao, L., Sun, B., & Wang, G. (2022) · Frontiers in Immunology · doi:10.3389/fimmu.2022.705472 · Cited by 57
Integration and Application of Clinical Practice Guidelines for the Diagnosis of Idiopathic Pulmonary Fibrosis and Fibrotic Hypersensitivity Pneumonitis
Marinescu, D.-C., Raghu, G., Rémy‐Jardin, M., Travis, W. D., Adegunsoye, A., Beasley, M. B., Chung, J. H., Churg, A., Cottin, V., Egashira, R., et al. (2022) · CHEST Journal · doi:10.1016/j.chest.2022.06.013 · Cited by 56
Tissue‐resident memory T cells and lung immunopathology
Cheon, I. S., Son, Y. M., & Sun, J. (2023) · Immunological Reviews · doi:10.1111/imr.13201 · Cited by 64
Cellular crosstalk in fibrosis: Insights into macrophage and fibroblast dynamics
Froom, Z. S. C. S., Callaghan, N. I., & Huyer, L. D. (2025) · Journal of Biological Chemistry · doi:10.1016/j.jbc.2025.110203 · Cited by 64
Demographic and clinical profile of idiopathic pulmonary fibrosis patients in Spain: the SEPAR National Registry
on behalf of the SEPAR-IPF National Registry, Fernández‐Fabrellas, E., Molina‐Molina, M., Soriano, J. B., Portal, J. A. R., Ancochea, J., Valenzuela, C., & Xaubet, A. (2019) · Respiratory Research · doi:10.1186/s12931-019-1084-0 · Cited by 57
Anti‐fibrotic effects of different sources of MSC in bleomycin‐induced lung fibrosis in C57BL6 male mice
Periera‐Simon, S., Xia, X., Catanuto, P., Coronado, R. E., Kurtzberg, J., Bellio, M. A., Lee, Y., Khan, A., Smith, R. D., Elliot, S. J., et al. (2020) · Respirology · doi:10.1111/resp.13928 · Cited by 55
Mangostanin Derivatives as Novel and Orally Active Phosphodiesterase 4 Inhibitors for the Treatment of Idiopathic Pulmonary Fibrosis with Improved Safety
Huang, Y., Deng, J., Tian, Y., Liang, J., Xie, X. S., Huang, Y., Zhu, J., Zhu, Z., Zhou, Q., He, X., et al. (2021) · Journal of Medicinal Chemistry · doi:10.1021/acs.jmedchem.1c01085 · Cited by 52
LOXL4, but not LOXL2, is the critical determinant of pathological collagen cross-linking and fibrosis in the lung
Ma, H., Li, Q., Wong, W. R., N’Diaye, E.-N., Caplazi, P., Bender, H. S., Huang, Z., Arlantico, A., Jeet, S., Wong, A., et al. (2023) · Science Advances · doi:10.1126/sciadv.adf0133 · Cited by 57
A cGAS-dependent response links DNA damage and senescence in alveolar epithelial cells: a potential drug target in IPF
Schuliga, M. J., Kanwal, A., Read, J. M., Blokland, K. E. C., Burgess, J. K., Prêle, C. M., Mutsaers, S. E., Grainge, C., Thomson, C., James, A., et al. (2021) · American Journal of Physiology-Lung Cellular and Molecular Physiology · doi:10.1152/ajplung.00574.2020 · Cited by 54
Spatially Resolved Deconvolution of the Fibrotic Niche in Lung Fibrosis
Eyres, M., Bell, J. A., Davies, E. R., Fabre, A., Alzetani, A., Jogai, S., Marshall, B. G., Johnston, D. A., Xu, Z., Fletcher, S., et al. (2022) · Cell Reports · doi:10.1016/j.celrep.2022.111230 · Cited by 54
Self-citation
The Genetic and Epigenetic Footprint in Idiopathic Pulmonary Fibrosis and Familial Pulmonary Fibrosis: A State-of-the-Art Review
Tirelli, C., Pesenti, C., Miozzo, M. R., Mondoni, M., Fontana, L., & Centanni, S. (2022) · Diagnostics · doi:10.3390/diagnostics12123107 · Cited by 55
New therapeutic strategies for IPF: Based on the “phagocytosis-secretion-immunization” network regulation mechanism of pulmonary macrophages
Liu, G., Zhai, H., Zhang, T., Li, S., Li, N., Chen, J., Gu, M., Qin, Z., & Liu, X. (2019) · Biomedicine & Pharmacotherapy · doi:10.1016/j.biopha.2019.109230 · Cited by 55
Improving the reactivity of hydrazine-bearing MRI probes for in vivo imaging of lung fibrogenesis
Akam, E. A., Abston, E. D., Rotile, N. J., Slattery, H., Zhou, I. Y., Lanuti, M. D., & Caravan, P. D. (2019) · Chemical Science · doi:10.1039/c9sc04821a · Cited by 51
Health-related quality of life and symptoms in patients with IPF treated with nintedanib: analyses of patient-reported outcomes from the INPULSIS® trials
Kreuter, M., Wuyts, W. A., Wijsenbeek, M. S., Bajwah, S., Maher, T. M., Stowasser, S., Male, N., Stansen, W., Schoof, N., Orsatti, L., et al. (2020) · Respiratory Research · doi:10.1186/s12931-020-1298-1 · Cited by 53
Atractylenolide III attenuates bleomycin-induced experimental pulmonary fibrosis and oxidative stress in rat model via Nrf2/NQO1/HO-1 pathway activation
Huai, B., & Ding, J. (2020) · Immunopharmacology and Immunotoxicology · doi:10.1080/08923973.2020.1806871 · Cited by 54
Endogenous tryptophan metabolite 5-Methoxytryptophan inhibits pulmonary fibrosis by downregulating the TGF-β/SMAD3 and PI3K/AKT signaling pathway
Fang, L., Chen, H., Kong, R., & Que, J. (2020) · Life Sciences · doi:10.1016/j.lfs.2020.118399 · Cited by 52
Extracellular vesicles derived from umbilical cord mesenchymal stromal cells alleviate pulmonary fibrosis by means of transforming growth factor-β signaling inhibition
Shi, L., Ren, J., Li, J., Wang, D., Wang, Y., Qin, T., Li, X., Zhang, G., Li, C., & Wang, Y. (2021) · Stem Cell Research & Therapy · doi:10.1186/s13287-021-02296-8 · Cited by 52
N6-Methyladenosine Modification of CDH1 mRNA Promotes PM2.5-Induced Pulmonary Fibrosis via Mediating Epithelial Mesenchymal Transition
Ning, J., Du, H., Zhang, Y., Liu, Q., Jiang, T., Pang, Y., Tian, X., Yan, L., Niu, Y., & Zhang, R. (2021) · Toxicological Sciences · doi:10.1093/toxsci/kfab133 · Cited by 52
Iron-Dependent Mitochondrial Dysfunction Contributes to the Pathogenesis of Pulmonary Fibrosis
Takahashi, M., Mizumura, K., Gon, Y., Shimizu, T., Kozu, Y., Shikano, S., Iida, Y., Hikichi, M., Okamoto, S., Tsuya, K., et al. (2022) · Frontiers in Pharmacology · doi:10.3389/fphar.2021.643980 · Cited by 53
Vascular remodelling in idiopathic pulmonary fibrosis patients and its detrimental effect on lung physiology: potential role of endothelial-to-mesenchymal transition
Gaikwad, A. V., Lu, W., Dey, S., Bhattarai, P., Chia, C., Larby, J., Haug, G., Myers, S., Jaffar, J., Westall, G. P., et al. (2022) · ERJ Open Research · doi:10.1183/23120541.00571-2021 · Cited by 52
Idiopathic pulmonary fibrosis (IPF): disease pathophysiology, targets, and potential therapeutic interventions
Saha, P., & Talwar, P. (2023) · Molecular and Cellular Biochemistry · doi:10.1007/s11010-023-04845-6 · Cited by 54
The Role of Immune Cells in the Pathogenesis of Idiopathic Pulmonary Fibrosis
Xu, Y., Lan, P., & Wang, T. (2023) · Medicina · doi:10.3390/medicina59111984 · Cited by 58
The IL-33 Receptor ST2 Regulates Pulmonary Inflammation and Fibrosis to Bleomycin
Fanny, M., Nascimento, M., Baron, L., Schricke, C., Maillet, I., Akbal, M., Riteau, N., Bert, M. L., Quesniaux, V. F. J., Ryffel, B., et al. (2018) · Frontiers in Immunology · doi:10.3389/fimmu.2018.01476 · Cited by 53
The Antifibrotic Activity of Prostacyclin Receptor Agonism Is Mediated through Inhibition of YAP/TAZ
Zmajkovicova, K., Menyhart, K., Bauer, Y., Studer, R. O., Renault, B., Schnoebelen, M., Bolinger, M., Nayler, O., & Gatfield, J. (2018) · American Journal of Respiratory Cell and Molecular Biology · doi:10.1165/rcmb.2018-0142oc · Cited by 56
The role of MIF in chronic lung diseases: looking beyond inflammation
Florez-Sampedro, L., Soto-Gamez, A., Poelarends, G. J., & Melgert, B. N. (2020) · American Journal of Physiology-Lung Cellular and Molecular Physiology · doi:10.1152/ajplung.00521.2019 · Cited by 47
Astragalus and its formulas as a therapeutic option for fibrotic diseases: Pharmacology and mechanisms
Zhu, Y., Chai, Y., Xiao, G., Liu, Y., Xie, X., Xiao, W. L., Zhou, P., Ma, W., Zhang, C., & Li, L. (2022) · Frontiers in Pharmacology · doi:10.3389/fphar.2022.1040350 · Cited by 53
Identifying potential biomarkers of idiopathic pulmonary fibrosis through machine learning analysis
Wu, Z., Chen, H., Ke, S., Mo, L., Qiu, M., Zhu, G., Zhu, W., & Liu, L. (2023) · Scientific Reports · doi:10.1038/s41598-023-43834-z · Cited by 53
The role of cGAS-STING signaling in pulmonary fibrosis and its therapeutic potential
Zhang, J., Zhang, L., Chen, Y., Fang, X., Li, B., & Mo, C. (2023) · Frontiers in Immunology · doi:10.3389/fimmu.2023.1273248 · Cited by 52
The autocrine CXCR4/CXCL12 axis contributes to lung fibrosis through modulation of lung fibroblast activity
Li, F., Xu, X., Geng, J., Wan, X., & Dai, H. (2020) · Experimental and Therapeutic Medicine · doi:10.3892/etm.2020.8433 · Cited by 51
Exosomal STIMATE derived from type II alveolar epithelial cells controls metabolic reprogramming of tissue-resident alveolar macrophages
Feng, Z., Zhou, J., Li, Q., Chu, L., Jiang, Y., Zhang, X., Yan, L., Liu, Y., Jiang, J. H., Xu, P., et al. (2023) · Theranostics · doi:10.7150/thno.82552 · Cited by 50
Incidence and prevalence of idiopathic pulmonary fibrosis: a systematic literature review and meta-analysis
Golchin, N., Patel, A., Scheuring, J., Wan, V., Hofer, K., Collet, J., Elpers, B., & Lesperance, T. (2025) · BMC Pulmonary Medicine · doi:10.1186/s12890-025-03836-1 · Cited by 50
Costunolide inhibits pulmonary fibrosis via regulating NF-kB and TGF-β1/Smad2/Nrf2-NOX4 signaling pathways
Liu, B., Rong, Y., Sun, D., Li, W., Chen, H., Cao, B., & Wang, T. (2019) · Biochemical and Biophysical Research Communications · doi:10.1016/j.bbrc.2019.01.104 · Cited by 50
Genome-Wide Association Study: Functional Variant rs2076295 Regulates Desmoplakin Expression in Airway Epithelial Cells
Hao, Y. T., Bates, S., Mou, H., Yun, J. H., Pham, B., Liu, J., Qiu, W., Guo, F., Morrow, J. D., Hersh, C. P., et al. (2020) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201910-1958oc · Cited by 47
Targeted Dual Small Interfering Ribonucleic Acid Delivery via Non‐Viral Polymeric Vectors for Pulmonary Fibrosis Therapy
Ji, Q., Hou, J., Yong, X., Gong, G., Muddassir, M., Tang, T., Xie, J., Fan, W., & Chen, X. S. (2021) · Advanced Materials · doi:10.1002/adma.202007798 · Cited by 49
Health repercussions of environmental exposure to lead: Methylation perspective
Shiek, S. S., Mani, M. S., Kabekkodu, S. P., & Dsouza, H. S. (2021) · Toxicology · doi:10.1016/j.tox.2021.152927 · Cited by 49
PD-L1 upregulation promotes drug-induced pulmonary fibrosis by inhibiting vimentin degradation
Li, Q., Deng, M., Wang, R., Luo, H., Luo, Y., Zhang, D.-D., Chen, K., Cao, X., Yang, G., Zhao, T., et al. (2022) · Pharmacological Research · doi:10.1016/j.phrs.2022.106636 · Cited by 50
Elevated expression of macrophage MERTK exhibits profibrotic effects and results in defective regulation of efferocytosis function in pulmonary fibrosis
She, Y., Xu, X., Yu, Q., Yang, X., He, J., & Tang, X. X. (2023) · Respiratory Research · doi:10.1186/s12931-023-02424-3 · Cited by 49
Exosomal miR-17-5p from human embryonic stem cells prevents pulmonary fibrosis by targeting thrombospondin-2
Liu, Q., Bi, Y., Song, S., Zhu, K., Qiao, X., Wang, H., & Ji, G. (2023) · Stem Cell Research & Therapy · doi:10.1186/s13287-023-03449-7 · Cited by 51
Clinical advances and challenges in targeting FGF/FGFR signaling in lung cancer
Peng, M., Deng, J., & Li, X. (2024) · Molecular Cancer · doi:10.1186/s12943-024-02167-9 · Cited by 49
Autophagy as a double-edged sword in pulmonary epithelial injury: a review and perspective
Li, Z., Wu, Y., Xu, X.-C., Zhou, J.-S., Wang, Y., Shen, H., & Chen, Z. (2017) · American Journal of Physiology-Lung Cellular and Molecular Physiology · doi:10.1152/ajplung.00562.2016 · Cited by 50
Efficacy and Safety of Nintedanib for the Treatment of Idiopathic Pulmonary Fibrosis: An Update
Rodríguez-Portal, J. A. (2017) · Drugs in R&D · doi:10.1007/s40268-017-0221-9 · Cited by 49
PDE4B inhibition by nerandomilast: Effects on lung fibrosis and transcriptome in fibrotic rats and on biomarkers in human lung epithelial cells
Reininger, D., Fundel‐Clemens, K., Mayr, C. H., Wollin, L., Laemmle, B., Quast, K., Nickolaus, P., & Herrmann, F. E. (2024) · British Journal of Pharmacology · doi:10.1111/bph.17303 · Cited by 48
Recent advances in vitamin D implications in chronic respiratory diseases
Gaudet, M., Plesa, M., Mogas, A. K., Jalaleddine, N., Hamid, Q. A., & Heialy, S. A. (2022) · Respiratory Research · doi:10.1186/s12931-022-02147-x · Cited by 48
A Causal Atlas on Comorbidities in Idiopathic Pulmonary Fibrosis
Zhu, J., Zhou, D., Wang, J., Yang, Y., Chen, D., He, F., & Li, Y. (2023) · CHEST Journal · doi:10.1016/j.chest.2023.02.038 · Cited by 48
Dynamic atlas of immune cells reveals multiple functional features of macrophages associated with progression of pulmonary fibrosis
Lv, J., Gao, H., Ma, J., Liu, J., Tian, Y., Yang, C., Li, M., Zhao, Y., Li, Z., Zhang, X., et al. (2023) · Frontiers in Immunology · doi:10.3389/fimmu.2023.1230266 · Cited by 48
Regulation of myofibroblast dedifferentiation in pulmonary fibrosis
Ju, X., Wang, K., Wang, C., Zeng, C., Wang, Y., & Yu, J. (2024) · Respiratory Research · doi:10.1186/s12931-024-02898-9 · Cited by 51
Regulatory T cells are a double-edged sword in pulmonary fibrosis
Wang, F., Xia, H., & Yao, S. (2020) · International Immunopharmacology · doi:10.1016/j.intimp.2020.106443 · Cited by 51
AXL Is a Potential Target for the Treatment of Intestinal Fibrosis
Steiner, C. A., Rodansky, E. S., Johnson, L. A., Berinstein, J. A., Cushing, K. C., Huang, S., Spence, J. R., & Higgins, P. (2020) · Inflammatory Bowel Diseases · doi:10.1093/ibd/izaa169 · Cited by 48
miR-21 in EVs from pulmonary epithelial cells promotes myofibroblast differentiation via glycolysis in arsenic-induced pulmonary fibrosis
Wang, P., Xiao, T., Li, J., Wang, D., Sun, J., Cheng, C., Ma, H., Xue, J., Li, Y., Zhang, A., et al. (2021) · Environmental Pollution · doi:10.1016/j.envpol.2021.117259 · Cited by 49
Nintedanib Inhibits Endothelial Mesenchymal Transition in Bleomycin-Induced Pulmonary Fibrosis via Focal Adhesion Kinase Activity Reduction
Yu, W.-K., Chen, W., Su, V. Y., Shen, H., Wu, H.-H., Chen, H., & Yang, K. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms23158193 · Cited by 48
IgA-producing B cells in lung homeostasis and disease
Bertrand, Y., Montalvo, A. S., Hox, V., Froidure, A., & Pilette, C. (2023) · Frontiers in Immunology · doi:10.3389/fimmu.2023.1117749 · Cited by 47
YTHDC1 delays cellular senescence and pulmonary fibrosis by activating ATR in an m6A-independent manner
Zhang, C., Chen, L., Xie, C., Wang, F., Wang, J., Zhou, H., Liu, Q., Zeng, Z., Li, N., Huang, J., et al. (2023) · The EMBO Journal · doi:10.1038/s44318-023-00003-2 · Cited by 48
Novel inhalation therapy in pulmonary fibrosis: principles, applications and prospects
Zheng, M., Zhu, W., Gao, F., Zhuo, Y., Mo, Z., Wu, G., & Feng, C. (2024) · Journal of Nanobiotechnology · doi:10.1186/s12951-024-02407-6 · Cited by 53
Insights into the Cellular and Molecular Mechanisms behind the Antifibrotic Effects of Nerandomilast
Reininger, D., Wolf, F., Mayr, C. H., Wespel, S. L., Laufhaeger, N., Geillinger‐Kästle, K. E., Dick, A., Gantner, F., Nickolaus, P., & Herrmann, F. E. (2025) · American Journal of Respiratory Cell and Molecular Biology · doi:10.1165/rcmb.2024-0614oc · Cited by 64
Multifaceted Role of Microbiota‐Derived Indole‐3‐Acetic Acid in Human Diseases and Its Potential Clinical Application
Shaheen, N., Miao, J., Xia, B., Zhao, Y., & Zhao, J. (2025) · The FASEB Journal · doi:10.1096/fj.202500295r · Cited by 49
Neutralization of IL-18 by IL-18 binding protein ameliorates bleomycin-induced pulmonary fibrosis via inhibition of epithelial-mesenchymal transition
Zhang, L., Zhang, Y., Chang, F., Zhang, J., Wang, L., Yi, Z.-W., & Gao, G. (2018) · Biochemical and Biophysical Research Communications · doi:10.1016/j.bbrc.2018.11.129 · Cited by 47
Dysregulation of club cell biology in idiopathic pulmonary fibrosis
Zuo, W.-L., Rostami, M. R., LeBlanc, M. G., Kaner, R. J., O’Beirne, S. L., Mezey, J. G., Leopold, P. L., Quast, K., Visvanathan, S., Fine, J. S., et al. (2020) · PLoS ONE · doi:10.1371/journal.pone.0237529 · Cited by 50
Bidirectional epithelial–mesenchymal crosstalk provides self-sustaining profibrotic signals in pulmonary fibrosis
Yao, L., Zhou, Y., Li, J., Wickens, L., Conforti, F., Rattu, A., Ibrahim, F. M., Alzetani, A., Marshall, B. G., Fletcher, S., et al. (2021) · Journal of Biological Chemistry · doi:10.1016/j.jbc.2021.101096 · Cited by 47
Self-citation
Systematic Review and Meta-analysis of Pirfenidone, Nintedanib, and Pamrevlumab for the Treatment of Idiopathic Pulmonary Fibrosis
Di Martino, E., Provenzani, A., Vitulo, P., & Polidori, P. (2021) · Annals of Pharmacotherapy · doi:10.1177/1060028020964451 · Cited by 46
Fibroblasts positive for meflin have anti-fibrotic properties in pulmonary fibrosis
Nakahara, Y., Hashimoto, N., Sakamoto, K., Enomoto, A., Adams, T. S., Yokoi, T., Omote, N., Poli, S., Ando, A., Wakahara, K., et al. (2021) · European Respiratory Journal · doi:10.1183/13993003.03397-2020 · Cited by 46
Mitophagy-Enhanced Nanoparticle-Engineered Mitochondria Restore Homeostasis of Mitochondrial Pool for Alleviating Pulmonary Fibrosis
Wang, Y., Hu, L., Liu, N.-H., Yang, J.-S., Xing, L., Jeong, J., Li, L., & Jiang, H. (2024) · ACS Nano · doi:10.1021/acsnano.4c10328 · Cited by 47
Best supportive care for idiopathic pulmonary fibrosis: current gaps and future directions
Ferrara, G., Luppi, F., Birring, S. S., Cerri, S., Caminati, A., Sköld, M. C., & Kreuter, M. (2018) · European Respiratory Review · doi:10.1183/16000617.0076-2017 · Cited by 47

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
ORCID: Richeldi, Luca
Collard, Harold R
Jones, Mark G
Venue: The Lancet
Publisher: Elsevier BV
Published: 2017-05
Volume: 389
Issue: 10082
Pages: 1941-1952
ISSN: 0099-5355 (ISSN-L)
0140-6736
DOI: 10.1016/s0140-6736(17)30866-8
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
Licence: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
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

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review

Idiopathic pulmonary fibrosis

Abstract

Overview

Integrity signals

Verification

Claims the paper makes

Claims other papers make about this one

Citations

Cites

Cited by

Related

Metadata

Record

About these checks