Idiopathic pulmonary fibrosis

Richeldi, Luca; Collard, Harold R; Jones, Mark G

doi:10.1016/s0140-6736(17)30866-8

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

Idiopathic pulmonary fibrosis

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

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

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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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IPF is marked by interstitial pneumonia, dyspnea and cough, and histologically characterized by fibroblast proliferation and extracellular matrix remodeling.
“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]
The median survival time after IPF diagnosis is 2-4 years despite widespread availability of targeted antifibrotic therapies.
“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]
Clinical trials of nintedanib and pirfenidone in IPF demonstrated only slowing of disease progression as measured by FVC and QOL, with unclear benefit to survival.
“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]
IPF is an age-related, progressive lung disease.
“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]
The Pdgf pathway is crucial to disease pathogenesis.
“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]

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du Bois, R. M., Weycker, D. A., Albera, C., Bradford, W. Z., Costabel, U., Kartashov, A. I., LANCASTER, L. H., Noble, P. W., Raghu, G., Sahn, S. A., et al. (2011) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201011-1790oc · Cited by 423 · see citing works
Anti-acid treatment and disease progression in idiopathic pulmonary fibrosis: an analysis of data from three randomised controlled trials
Lee, J. S., Collard, H. R., Anstrom, K. J., Martínez, F. J., Noth, I., Roberts, R., Yow, E. S., & Raghu, G. (2013) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(13)70105-x · Cited by 411 · see citing works
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Lung microbiome and disease progression in idiopathic pulmonary fibrosis: an analysis of the COMET study
Han, M. K., Zhou, Y., Murray, S., Tayob, N., Noth, I., Lama, V. N., Moore, B. B., White, E. S., Flaherty, K. R., Hu, B., et al. (2014) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(14)70069-4 · Cited by 408 · see citing works
Role of Lung Pericytes and Resident Fibroblasts in the Pathogenesis of Pulmonary Fibrosis
Hung, C. F., Linn, G. R., Chow, Y., Kobayashi, A., Mittelsteadt, K. L., Altemeier, W. A., Gharib, S. A., Schnapp, L. M., & Duffield, J. S. (2013) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201212-2297oc · Cited by 388 · see citing works
Is Idiopathic Pulmonary Fibrosis an Environmental Disease?
Taskar, V. S., & Coultas, D. B. (2006) · Proceedings of the American Thoracic Society · doi:10.1513/pats.200512-131tk · Cited by 387 · see citing works
Inhibition of PDGF, VEGF and FGF signalling attenuates fibrosis
Chaudhary, N. I., Roth, G. J., Hilberg, F., Müller–Quernheim, J., Prasse, A., Zissel, G., Schnapp, A., & Park, J. E. (2007) · European Respiratory Journal · doi:10.1183/09031936.00152106 · Cited by 386 · see citing works
Imatinib Treatment for Idiopathic Pulmonary Fibrosis: Randomized Placebo-controlled Trial Results
Daniels, C. E., Lasky, J. A., Limper, A. H., Mieras, K., Gabor, E., & Schroeder, D. R. (2010) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200906-0964oc · Cited by 373 · see citing works
TOLLIP , MUC5B , and the Response to N -Acetylcysteine among Individuals with Idiopathic Pulmonary Fibrosis
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Bronchoscopic Lung Cryobiopsy Increases Diagnostic Confidence in the Multidisciplinary Diagnosis of Idiopathic Pulmonary Fibrosis
Tomassetti, S., Wells, A. U., Costabel, U., Cavazza, A., Colby, T. V., Rossi, G., Sverzellati, N., Carloni, A., Carretta, E., Buccioli, M., et al. (2015) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201504-0711oc · Cited by 338 · see citing works
Interobserver agreement for the ATS/ERS/JRS/ALAT criteria for a UIP pattern on CT
Walsh, S. L. F., Calandriello, L., Sverzellati, N., Wells, A. U., & Hansell, D. M. (2015) · Thorax · doi:10.1136/thoraxjnl-2015-207252 · Cited by 336 · see citing works
Serum CC-Chemokine Ligand 18 Concentration Predicts Outcome in Idiopathic Pulmonary Fibrosis
Prasse, A., Probst, C., Bargagli, E., Zissel, G., Toews, G. B., Flaherty, K. R., Olschewski, M., Rottoli, P., & Müller–Quernheim, J. (2009) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200808-1201oc · Cited by 329 · see citing works
Peripheral blood mononuclear cell gene expression profiles predict poor outcome in idiopathic pulmonary fibrosis
Herazo-Maya, J. D., Noth, I., Duncan, S. R., Kim, S., Ma, S.-F., Tseng, G. C., Feingold, E., Juan-Guardela, B. M., Richards, T. J., Lussier, Y., et al. (2013) · Science Translational Medicine · doi:10.1126/scitranslmed.3005964 · Cited by 325 · see citing works
Obstructive Sleep Apnea Is Common in Idiopathic Pulmonary Fibrosis
LANCASTER, L. H., Mason, W. R., Parnell, J. A., Rice, T. W., Loyd, J. E., Milstone, A. P., Collard, H. R., & Malow, B. A. (2009) · CHEST Journal · doi:10.1378/chest.08-2776 · Cited by 320 · see citing works
Self-citation
Radiologic Findings Are Strongly Associated With a Pathologic Diagnosis of Usual Interstitial Pneumonia
Hunninghake, G. W., Lynch, D. A., Galvin, J. R., Gross, B. H., ller, N. L. M., Schwartz, D. A., King, T. E., Lynch, J. P., Hegele, R. G., Waldron, J. A., et al. (2003) · CHEST Journal · doi:10.1378/chest.124.4.1215 · Cited by 318 · see citing works
Forced Vital Capacity in Idiopathic Pulmonary Fibrosis — FDA Review of Pirfenidone and Nintedanib
Karimi-Shah, B. A., & Chowdhury, B. A. (2015) · New England Journal of Medicine · doi:10.1056/nejmp1500526 · Cited by 316 · see citing works
Correction: Corrigendum: Elucidating the role of disorder and free-carrier recombination kinetics in CH3NH3PbI3 perovskite films
La‐o‐vorakiat, C., Salim, T., Kadro, J. M., Khuc, M.-T., Haselsberger, R., Cheng, L., Xia, H., Gurzadyan, G. G., Su, H., Lam, Y. M., et al. (2016) · Nature Communications · doi:10.1038/ncomms11054 · Cited by 316 · see citing works
Longitudinal change in collagen degradation biomarkers in idiopathic pulmonary fibrosis: an analysis from the prospective, multicentre PROFILE study
Jenkins, R. G., Simpson, J. K., Saini, G., Bentley, J. H., Russell, A.-M., Braybrooke, R., Molyneaux, P. L., McKeever, T. M., Wells, A. U., Flynn, A., et al. (2015) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(15)00048-x · Cited by 314 · see citing works
Delayed Access and Survival in Idiopathic Pulmonary Fibrosis: A Cohort Study
Lamas, D. J., Kawut, S. M., Bagiella, E., Philip, N., Arcasoy, S. M., & Lederer, D. J. (2011) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201104-0668oc · Cited by 314 · see citing works
Prevalence and prognosis of unclassifiable interstitial lung disease
Ryerson, C. J., Urbania, T. H., Richeldi, L., Mooney, J. J., Lee, J. S., Jones, K. D., Elicker, B. M., Koth, L. L., King, T. E., Wolters, P. J., et al. (2012) · European Respiratory Journal · doi:10.1183/09031936.00131912 · Cited by 312 · see citing works
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Number and Proliferation of Basal and Parabasal Cells in Normal Human Airway Epithelium
Boers, J. E., Ambergen, A. W., & Thunnissen, E. (1998) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/ajrccm.157.6.9707011 · Cited by 287 · see citing works
Hyaluronan and TLR4 promote surfactant-protein-C-positive alveolar progenitor cell renewal and prevent severe pulmonary fibrosis in mice
Liang, J., Zhang, Y., Xie, T., Liu, N., Chen, H., Geng, Y., Kurkciyan, A., Mena, J. M., Stripp, B. R., Jiang, D., et al. (2016) · Nature Medicine · doi:10.1038/nm.4192 · Cited by 287 · see citing works
Efficacy of Nintedanib in Idiopathic Pulmonary Fibrosis across Prespecified Subgroups in INPULSIS
Costabel, U., Inoue, Y., Richeldi, L., Collard, H. R., Tschoepe, I., Stowasser, S., & Azuma, A. (2016) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201503-0562oc · Cited by 280 · see citing works
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Macitentan for the treatment of idiopathic pulmonary fibrosis: the randomised controlled MUSIC trial
Raghu, G., Million-Rousseau, R., Morganti, A., Perchenet, L., Behr, J., & the MUSIC Study Group (2013) · European Respiratory Journal · doi:10.1183/09031936.00104612 · Cited by 277 · see citing works
Telomere dysfunction in alveolar epithelial cells causes lung remodeling and fibrosis
Naikawadi, R. P., Disayabutr, S., Mallavia, B., Donne, M. L., Green, G., La, J. L., Rock, J. R., Looney, M. R., & Wolters, P. J. (2016) · JCI Insight · doi:10.1172/jci.insight.86704 · Cited by 267 · see citing works
Histopathologic Features and Outcome of Patients With Acute Exacerbation of Idiopathic Pulmonary Fibrosis Undergoing Surgical Lung Biopsy
Parambil, J. G., Myers, J. L., & Ryu, J. H. (2005) · CHEST Journal · doi:10.1378/chest.128.5.3310 · Cited by 258 · see citing works
Expression of cilium-associated genes defines novel molecular subtypes of idiopathic pulmonary fibrosis
Yang, I. V., Coldren, C. D., Leach, S. M., Seibold, M. A., Murphy, E., Lin, J., Rosen, R. Z., Neidermyer, A. J., McKean, D. F., Groshong, S. D., et al. (2013) · Thorax · doi:10.1136/thoraxjnl-2012-202943 · Cited by 244 · see citing works
Clinical Predictors of a Diagnosis of Idiopathic Pulmonary Fibrosis
Fell, C. D., Martínez, F. J., Liu, L. X., Murray, S., Han, M. K., Kazerooni, E. A., Gross, B. H., Myers, J. L., Travis, W. D., Colby, T. V., et al. (2010) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200906-0959oc · Cited by 243 · see citing works
Idiopathic Pulmonary Fibrosis: A Genetic Disease That Involves Mucociliary Dysfunction of the Peripheral Airways
Evans, C. M., Fingerlin, T. E., Schwarz, M. I., Lynch, D. A., Kurche, J. S., Warg, L. A., Yang, I. V., & Schwartz, D. A. (2016) · Physiological Reviews · doi:10.1152/physrev.00004.2016 · Cited by 233 · see citing works
Mucin 5B promoter polymorphism is associated with idiopathic pulmonary fibrosis but not with development of lung fibrosis in systemic sclerosis or sarcoidosis
Stock, C. J. W., Sato, H., Fonseca, C., BANYA, W. A. S., Molyneaux, P. L., Adamali, H. I., Russell, A., Denton, C. P., Abraham, D., Hansell, D. M., et al. (2013) · Thorax · doi:10.1136/thoraxjnl-2012-201786 · Cited by 231 · see citing works
Antacid therapy and disease outcomes in idiopathic pulmonary fibrosis: a pooled analysis
Kreuter, M., Wuyts, W. A., Renzoni, E. A., Koschel, D., Maher, T. M., Kolb, M. R. J., Weycker, D. A., Spagnolo, P., Kirchgaessler, K.-U., Herth, F. J. F., et al. (2016) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(16)00067-9 · Cited by 216 · see citing works
Diagnostic Yield and Complications of Transbronchial Lung Cryobiopsy for Interstitial Lung Disease. A Systematic Review and Metaanalysis
Johannson, K. A., Marcoux, V. S., Ronksley, P. E., & Ryerson, C. J. (2016) · Annals of the American Thoracic Society · doi:10.1513/annalsats.201606-461sr · Cited by 216 · see citing works
A Study of the Cough Reflex in Idiopathic Pulmonary Fibrosis
Hope-Gill, B., Hilldrup, S., Davies, C., Newton, R. P., & Harrison, N. K. (2003) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200304-597oc · Cited by 215 · see citing works
Safety and pharmacokinetics of nintedanib and pirfenidone in idiopathic pulmonary fibrosis
Ogura, T., Taniguchi, H., Azuma, A., Inoue, Y., Kondoh, Y., Hasegawa, Y., Bando, M., Abe, S., Mochizuki, Y., Chida, K., et al. (2015) · European Respiratory Journal · doi:10.1183/09031936.00198013 · Cited by 215 · see citing works
A Variant in the Promoter of MUC5B and Idiopathic Pulmonary Fibrosis
Zhang, Y., Noth, I., Garcia, J. G. N., & Kaminski, N. (2011) · New England Journal of Medicine · doi:10.1056/nejmc1013504 · Cited by 214 · see citing works
Abnormal Re-epithelialization and Lung Remodeling in Idiopathic Pulmonary Fibrosis: The Role of ΔN-p63
Chilosi, M., Poletti, V. E., Murer, B., Lestani, M., Cancellieri, A., Montagna, L., Piccoli, P., Cangi, M. G., Semenzato, G., & Doglioni, C. (2002) · Laboratory Investigation · doi:10.1097/01.lab.0000032380.82232.67 · Cited by 212 · see citing works
Does Chronic Microaspiration Cause Idiopathic Pulmonary Fibrosis?
Lee, J. S., Collard, H. R., Raghu, G., Sweet, M. P., Hays, S. R., Campos, G. M., Golden, J. A., & King, T. E. (2010) · The American Journal of Medicine · doi:10.1016/j.amjmed.2009.07.033 · Cited by 209 · see citing works
Self-citation
Rare Variants in RTEL1 Are Associated with Familial Interstitial Pneumonia
Cogan, J. D., Kropski, J. A., Zhao, M., Mitchell, D. B., Rives, L. C., Markin, C. R., Garnett, E. T., Montgomery, K. H., Mason, W. R., McKean, D. F., et al. (2015) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201408-1510oc · Cited by 197 · see citing works
Fibroblast Foci Are Not Discrete Sites of Lung Injury or Repair
Cool, C. D., Groshong, S. D., Pradeep, R., Henson, P. M., Stewart, J., & Brown, K. K. (2006) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200602-205oc · Cited by 196 · see citing works
Treating idiopathic pulmonary fibrosis with the addition of co-trimoxazole: a randomised controlled trial
Shulgina, L., Cahn, A. P., Chilvers, E. R., Parfrey, H., Clark, A., Wilson, E., Twentyman, O. P., Davison, A. G., Curtin, J. J., Crawford, M., et al. (2012) · Thorax · doi:10.1136/thoraxjnl-2012-202403 · Cited by 190 · see citing works
The Toll-like Receptor 3 L412F Polymorphism and Disease Progression in Idiopathic Pulmonary Fibrosis
O’Dwyer, D. N., Armstrong, M. E., Trujillo, G., Cooke, G., Keane, M. P., Fallon, P. G., Simpson, A. J., Millar, A., McGrath, E. E., Whyte, M. K. B., et al. (2013) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201304-0760oc · Cited by 184 · see citing works
Epidemiology and survival of idiopathic pulmonary fibrosis from national data in Canada
Hopkins, R. B., Burke, N., Fell, C. D., Dion, G., & Kolb, M. R. J. (2016) · European Respiratory Journal · doi:10.1183/13993003.01504-2015 · Cited by 181 · see citing works
Effect of nintedanib in subgroups of idiopathic pulmonary fibrosis by diagnostic criteria
Raghu, G., Wells, A. U., Nicholson, A. G., Richeldi, L., Flaherty, K. R., Le Maulf, F., Stowasser, S., Schlenker-Herceg, R., & Hansell, D. M. (2017) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201602-0402oc · Cited by 180 · see citing works
Self-citation
Effect of continued treatment with pirfenidone following clinically meaningful declines in forced vital capacity: analysis of data from three phase 3 trials in patients with idiopathic pulmonary fibrosis
Nathan, S. D., Albera, C., Bradford, W. Z., Costabel, U., du Bois, R. M., Fagan, E., Fishman, R. S., Glaspole, I., Glassberg, M. K., Glasscock, K. F., et al. (2016) · Thorax · doi:10.1136/thoraxjnl-2015-207011 · Cited by 179 · see citing works
Extensive Phenotyping of Individuals at Risk for Familial Interstitial Pneumonia Reveals Clues to the Pathogenesis of Interstitial Lung Disease
Kropski, J. A., Pritchett, J. M., Zoz, D. F., Crossno, P. F., Markin, C. R., Garnett, E. T., Degryse, A. L., Mitchell, D. B., Polosukhin, V. V., Rickman, O. B., et al. (2014) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201406-1162oc · Cited by 178 · see citing works
Telomeropathies: An emerging spectrum disorder
Holohan, B., Wright, W. E., & Shay, J. W. (2014) · Journal of Cell Biology · doi:10.1083/jcb.201401012 · Cited by 175 · see citing works
Pulmonary rehabilitation improves long-term outcomes in interstitial lung disease: A prospective cohort study
Ryerson, C. J., Cayou, C., Topp, F. J., Hilling, L., Camp, P. G., Wilcox, P., Khalil, N., Collard, H. R., & Garvey, C. (2013) · Respiratory Medicine · doi:10.1016/j.rmed.2013.11.016 · Cited by 173 · see citing works
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Pulmonary Rehabilitation in Interstitial Lung Disease
Koltharkar, M. (2021) · International Journal of Cardiovascular Research · Cited by 173 · see citing works
Efficacy of pirfenidone in patients with idiopathic pulmonary fibrosis with more preserved lung function
Albera, C., Costabel, U., Fagan, E., Glassberg, M. K., Gorina, E., LANCASTER, L. H., Lederer, D. J., Nathan, S. D., Spirig, D., & Swigris, J. J. (2016) · European Respiratory Journal · doi:10.1183/13993003.01966-2015 · Cited by 165 · see citing works
Understanding and managing toxicities of vascular endothelial growth factor (VEGF) inhibitors
Schmidinger, M. (2013) · European Journal of Cancer Supplements · doi:10.1016/j.ejcsup.2013.07.016 · Cited by 159 · see citing works
Genetic studies provide clues on the pathogenesis of idiopathic pulmonary fibrosis
Kropski, J. A., Lawson, W. E., Young, L. R., & Blackwell, T. S. (2012) · Disease Models & Mechanisms · doi:10.1242/dmm.010736 · Cited by 154 · see citing works
Mechanosensing by the α6-integrin confers an invasive fibroblast phenotype and mediates lung fibrosis
Chen, H., Qu, J., Huang, X., Kurundkar, A., Zhu, L., Yang, N., Venado, A. E., Ding, Q., Liu, G., Antony, V. B., et al. (2016) · Nature Communications · doi:10.1038/ncomms12564 · Cited by 153 · see citing works
Lung transplantation in idiopathic pulmonary fibrosis: a systematic review of the literature
Kistler, K. D., Nalysnyk, L., Rotella, P., & Esser, D. (2014) · BMC Pulmonary Medicine · doi:10.1186/1471-2466-14-139 · Cited by 151 · see citing works
Epithelial-Mesenchymal Interactions in Pulmonary Fibrosis
Horowitz, J. C., & Thannickal, V. J. (2006) · Seminars in Respiratory and Critical Care Medicine · doi:10.1055/s-2006-957332 · Cited by 146 · see citing works
Safety and tolerability of acetylcysteine and pirfenidone combination therapy in idiopathic pulmonary fibrosis: a randomised, double-blind, placebo-controlled, phase 2 trial
Behr, J., Bendstrup, E., Crestani, B., Günther, A., Olschewski, H., Sköld, C. M., Wells, A., Wuyts, W., Koschel, D., Kreuter, M., et al. (2016) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(16)30044-3 · Cited by 146 · see citing works
Aberrant expression and activity of histone deacetylases in sporadic idiopathic pulmonary fibrosis
Korfei, M., Skwarna, S., Henneke, I., MacKenzie, B., Klymenko, O., Saito, S., Ruppert, C., von der Beck, D., Mahavadi, P., Klepetko, W., et al. (2015) · Thorax · doi:10.1136/thoraxjnl-2014-206411 · Cited by 143 · see citing works
The impact of emphysema in pulmonary fibrosis
Cottin, V. (2013) · European Respiratory Review · doi:10.1183/09059180.00000813 · Cited by 138 · see citing works
The MUC5B Promoter Polymorphism Is Associated With Idiopathic Pulmonary Fibrosis in a Mexican Cohort but Is Rare Among Asian Ancestries
Peljto, A. L., Selman, M., Kim, D. S., Murphy, E., Tucker, L., Pardo, A., Lee, J. S., Ji, W., Schwarz, M. I., Yang, I. V., et al. (2014) · CHEST Journal · doi:10.1378/chest.14-0867 · Cited by 135 · see citing works
The use of pretest probability increases the value of high-resolution CT in diagnosing usual interstitial pneumonia
Brownell, R., Moua, T., Henry, T. S., Elicker, B. M., White, D. B., Vittinghoff, E., Jones, K. D., Urisman, A., Aravena, C., Johannson, K. A., et al. (2017) · Thorax · doi:10.1136/thoraxjnl-2016-209671 · Cited by 128 · see citing works
Self-citation
Expression of the developmental Sonic hedgehog (Shh) signalling pathway is up‐regulated in chronic lung fibrosis and the Shh receptor patched 1 is present in circulating T lymphocytes
Stewart, G. A., Hoyne, G. F., Ahmad, S. A., Jarman, E., Wallace, W. A., Harrison, D. J., Haslett, C., Lamb, J. R., & Howie, S. (2003) · The Journal of Pathology · doi:10.1002/path.1295 · Cited by 125 · see citing works
Pro-arrhythmic and pro-ischaemic effects of inhaled anticholinergic medications
Singh, S., Loke, Y. K., Enright, P., & Furberg, C. D. (2012) · Thorax · doi:10.1136/thoraxjnl-2011-201275 · Cited by 121 · see citing works
MUC5B Promoter Variant rs35705950 Affects MUC5B Expression in the Distal Airways in Idiopathic Pulmonary Fibrosis
Nakano, Y., Yang, I. V., Walts, A. D., Watson, A. M., Helling, B. A., Fletcher, A. A., Lara, A., Schwarz, M. I., Evans, C. M., & Schwartz, D. A. (2016) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201509-1872le · Cited by 113 · see citing works
Benefits of Pulmonary Rehabilitation in Idiopathic Pulmonary Fibrosis
Swigris, J. J., Fairclough, D. L., Morrison, M., Make, B. J., Kozora, E., Brown, K. K., & Wamboldt, F. S. (2011) · Respiratory Care · doi:10.4187/respcare.00939 · Cited by 104 · see citing works
Interventions to improve symptoms and quality of life of patients with fibrotic interstitial lung disease: a systematic review of the literature
Bajwah, S., Ross, J. R., Peacock, J. L., Higginson, I. J., Wells, A. U., Patel, A. S., Koffman, J., & Riley, J. L. (2012) · Thorax · doi:10.1136/thoraxjnl-2012-202040 · Cited by 101 · see citing works
Pirfenidone: A Novel Pharmacological Agent That Inhibits Leiomyoma Cell Proliferation and Collagen Production
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Specialist Palliative Care is More Than Drugs: A Retrospective Study of ILD Patients
Bajwah, S., Higginson, I. J., Ross, J. R., Wells, A. U., Birring, S. S., Patel, A. S., & Riley, J. L. (2011) · Lung · doi:10.1007/s00408-011-9355-7 · Cited by 100 · see citing works
An investigator‐driven study of everolimus in surgical lung biopsy confirmed idiopathic pulmonary fibrosis
Malouf, M. A., Hopkins, P. M.-A., Snell, G. I., & Glanville, A. R. (2011) · Respirology · doi:10.1111/j.1440-1843.2011.01955.x · Cited by 100 · see citing works
Three-dimensional characterization of fibroblast foci in idiopathic pulmonary fibrosis
Jones, M. G., Fabre, A., Schneider, P., Cinetto, F., Sgalla, G., Mavrogordato, M., Jogai, S., Alzetani, A., Marshall, B. G., O’Reilly, K. M., et al. (2016) · JCI Insight · doi:10.1172/jci.insight.86375 · Cited by 96 · see citing works
Self-citation
Genetic Evaluation and Testing of Patients and Families with Idiopathic Pulmonary Fibrosis
Kropski, J. A., Young, L. R., Cogan, J. D., Mitchell, D. B., LANCASTER, L. H., Worrell, J. A., Markin, C. R., Liu, N., Mason, W. R., Fingerlin, T. E., et al. (2016) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201609-1820pp · Cited by 92 · see citing works
Matrix, Mesenchyme, and Mechanotransduction
Tschumperlin, D. J. (2015) · Annals of the American Thoracic Society · doi:10.1513/annalsats.201407-320mg · Cited by 65 · see citing works
Laparoscopic anti-reflux surgery for idiopathic pulmonary fibrosis at a single centre
Raghu, G., Morrow, E. H., Collins, B. F., Ho, L. A., Hinojosa, M. W., Hayes, J. M., Spada, C. A., Oelschlager, B. K., Li, C., Yow, E. S., et al. (2016) · European Respiratory Journal · doi:10.1183/13993003.00488-2016 · Cited by 54 · see citing works
A double blind randomised placebo controlled pilot study of oral co-trimoxazole in advanced fibrotic lung disease
Varney, V., Parnell, H., Salisbury, D., Ratnatheepan, S., & Tayar, R. (2007) · Pulmonary Pharmacology & Therapeutics · doi:10.1016/j.pupt.2007.02.001 · Cited by 46 · see citing works
Occupational dust exposure and the aetiology of cryptogenic fibrosing alveolitis
Hubbard, R. (2001) · European Respiratory Journal · doi:10.1183/09031936.01.18s320119 · Cited by 39 · see citing works
Familial Interstitial Pneumonia
Talbert, J. L., Schwartz, D. A., & Steele, M. P. (2014) · Clinical Pulmonary Medicine · doi:10.1097/cpm.0000000000000034 · Cited by 11 · see citing works
Therapy for idiopathic pulmonary fibrosis: lessons from pooled data analyses
Troy, L. K., & Corte, T. J. (2015) · European Respiratory Journal · doi:10.1183/13993003.01669-2015 · Cited by 9 · see citing works

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Reference-based analysis of lung single-cell sequencing reveals a transitional profibrotic macrophage
Aran, D., Looney, A. P., Liu, L., Wu, E., Fong, V., Hsu, A., Chak, S., Naikawadi, R. P., Wolters, P. J., Abate, A. R., et al. (2019) · Nature Immunology · doi:10.1038/s41590-018-0276-y · Cited by 4,984 · see citing works
Wnt/β-catenin signalling: function, biological mechanisms, and therapeutic opportunities
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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

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