Long-term safety of pirfenidone: results of the prospective, observational PASSPORT study

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 · see citing works

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 · see citing works

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 · see citing works

Clinical course and prediction of survival in idiopathic pulmonary fibrosis

Ley, B., Collard, H. R., & King, T. E. (2011) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201006-0894ci · Cited by 1,765 · see citing works

Idiopathic pulmonary fibrosis

Meltzer, E. B., & Noble, P. W. (2008) · Orphanet Journal of Rare Diseases · doi:10.1186/1750-1172-3-8 · Cited by 415 · see citing works

Pirfenidone safety and adverse event management in idiopathic pulmonary fibrosis

Lancaster, L. H., de Andrade, J. A., Zibrak, J. D., Padilla, M. L., Albera, C., Nathan, S. D., Wijsenbeek, M. S., Stauffer, J. L., Kirchgaessler, K.-U., & Costabel, U. (2017) · European Respiratory Review · doi:10.1183/16000617.0057-2017 · Cited by 295 · see citing works

Pirfenidone and nintedanib for pulmonary fibrosis in clinical practice: Tolerability and adverse drug reactions

Galli, J. A., Pandya, A., Vega‐Olivo, M., Dass, C. A., Zhao, H., & Criner, G. J. (2017) · Respirology · doi:10.1111/resp.13024 · Cited by 258 · see citing works

Beyond the Randomized Clinical Trial

Nallamothu, B. K., Hayward, R. A., & Bates, E. (2008) · Circulation · doi:10.1161/circulationaha.107.703579 · Cited by 251 · see citing works

Randomized controlled trials and real life studies. Approaches and methodologies: a clinical point of view.

Saturni, S., Bellini, F., Braido, F., Paggiaro, P., Sanduzzi, A., Scichilone, N., Santus, P. A., Morandi, L., & Papi, A. (2014) · Pulmonary Pharmacology & Therapeutics · doi:10.1016/j.pupt.2014.01.005 · Cited by 245 · see citing works

Real World Experiences: Pirfenidone and Nintedanib are Effective and Well Tolerated Treatments for Idiopathic Pulmonary Fibrosis

Hughes, G., Toellner, H., Morris, H. G., Leonard, C. T., & Chaudhuri, N. (2016) · Journal of Clinical Medicine · doi:10.3390/jcm5090078 · Cited by 167 · see citing works

Pirfenidone in Idiopathic Pulmonary Fibrosis: Expert Panel Discussion on the Management of Drug-Related Adverse Events

Costabel, U., Bendstrup, E. M., Cottin, V., Dewint, P., Egan, J. J., Ferguson, J., Groves, R. W., Hellström, P. M., Kreuter, M., Maher, T. M., et al. (2014) · Advances in Therapy · doi:10.1007/s12325-014-0112-1 · Cited by 138 · see citing works

Safety of pirfenidone in patients with idiopathic pulmonary fibrosis: integrated analysis of cumulative data from 5 clinical trials

Lancaster, L., Albera, C., Bradford, W. Z., Costabel, U., du Bois, R. M., Fagan, E. A., Fishman, R. S., Glaspole, I., Glassberg, M. K., King, T. E., et al. (2016) · BMJ Open Respiratory Research · doi:10.1136/bmjresp-2015-000105 · Cited by 132 · see citing works

Erratum to: Pirfenidone in Idiopathic Pulmonary Fibrosis: Expert Panel Discussion on the Management of Drug-Related Adverse Events

Costabel, U., Bendstrup, E. M., Cottin, V., Dewint, P., Egan, J. J., Ferguson, J., Groves, R. W., Hellström, P. M., Kreuter, M., Maher, T. M., et al. (2014) · Advances in Therapy · doi:10.1007/s12325-014-0118-8 · Cited by 75 · see citing works

Pirfenidone Post-Authorization Safety Registry (PASSPORT): Update and Concomitant Use of NAC and/or Corticosteroids

Cottin, V., Maher, T. M., Azuma, A., Groves, L., Hormel, P., Sköld, M. C., Tomassetti, S., & Koschel, D. (2015) · CHEST Journal · doi:10.1378/chest.2245542 · Cited by 2 · see citing works

Antifibrotic therapy for idiopathic pulmonary fibrosis: time to treat

Maher, T. M., & Strek, M. E. (2019) · Respiratory Research · doi:10.1186/s12931-019-1161-4 · Cited by 266 · see citing works

Pharmacotherapy and adjunctive treatment for idiopathic pulmonary fibrosis (IPF)

Saito, S., Alkhatib, A. L., Kolls, J. K., Kondoh, Y., & Lasky, J. A. (2019) · Journal of Thoracic Disease · doi:10.21037/jtd.2019.04.62 · Cited by 148 · see citing works

Molecular Mechanisms and Cellular Contribution from Lung Fibrosis to Lung Cancer Development

Samarelli, A. V., Masciale, V., Aramini, B., Coló, G. P., Tonelli, R., Marchioni, A., Bruzzi, G., Gozzi, F., Andrisani, D., Castaniere, I., et al. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms222212179 · Cited by 92 · see citing works

Bexotegrast in Patients with Idiopathic Pulmonary Fibrosis: The INTEGRIS-IPF Clinical Trial

Lancaster, L., Cottin, V., Ramaswamy, M., Wuyts, W. A., Jenkins, R. G., Scholand, M. B., Kreuter, M., Valenzuela, C., Ryerson, C. J., Goldin, J., et al. (2024) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202403-0636oc · Cited by 80 · see citing works

Safety and efficacy of pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis and carrying a telomere-related gene mutation

Justet, A., Klay, D., Porcher, R., Cottin, V., Ahmad, K., Molina‐Molina, M., Nunès, H., Reynaud‐Gaubert, M., Naccache, J. M., Manali, E. D., et al. (2020) · European Respiratory Journal · doi:10.1183/13993003.03198-2020 · Cited by 76 · see citing works

Pirfenidone is a cardioprotective drug: Mechanisms of action and preclinical evidence

Aimo, A., Cerbai, E., Bartolucci, G., Adamo, L., Barison, A., Surdo, G. L., Biagini, S., Passino, C., & Emdin, M. (2020) · Pharmacological Research · doi:10.1016/j.phrs.2020.104694 · Cited by 74 · see citing works

TGF-β Pathway in Salivary Gland Fibrosis

Zhang, X., Yun, J. S., Han, D., Yook, J. I., Kim, H. S., & Cho, E. S. (2020) · International Journal of Molecular Sciences · doi:10.3390/ijms21239138 · Cited by 63 · see citing works

Efficacy and Safety of Admilparant, an LPA1 Antagonist, in Pulmonary Fibrosis: A Phase 2 Randomized Clinical Trial.

Corte, T. J., Behr, J., Cottin, V., Glassberg, M. K., Kreuter, M., Martinez, F. J., Ogura, T., Suda, T., Wijsenbeek, M., Berkowitz, E., et al. (2025) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202405-0977oc · Cited by 70 · see citing works

Real-world safety and effectiveness of pirfenidone and nintedanib in the treatment of idiopathic pulmonary fibrosis: a systematic review and meta-analysis

Kou, M., Jiao, Y., Li, Z., Wei, B., Li, Y., Cai, Y., & Wei, W. (2024) · European Journal of Clinical Pharmacology · doi:10.1007/s00228-024-03720-7 · Cited by 60 · see citing works

Patient gender bias on the diagnosis of idiopathic pulmonary fibrosis

Assayag, D., Morisset, J., Johannson, K. A., Wells, A. U., & Walsh, S. L. F. (2020) · Thorax · doi:10.1136/thoraxjnl-2019-213968 · Cited by 54 · see citing works

Treatment of idiopathic pulmonary fibrosis and progressive pulmonary fibrosis: A position statement from the Thoracic Society of Australia and New Zealand 2023 revision

Mackintosh, J. A., Keir, G. J., Troy, L. K., Holland, A. E., Grainge, C., Chambers, D. C., Sandford, D., Jo, H. E., Glaspole, I., WILSHER, M. L., et al. (2024) · Respirology · doi:10.1111/resp.14656 · Cited by 54 · see citing works

Antifibrotic Drug Use in Patients with Idiopathic Pulmonary Fibrosis. Data from the IPF-PRO Registry

Salisbury, M. L., Conoscenti, C., Culver, D. A., Yow, E. S., Neely, M. L., Bender, S. D., Hartmann, N., Palmer, S. M., & Leonard, T. B. (2020) · Annals of the American Thoracic Society · doi:10.1513/annalsats.201912-880oc · Cited by 53 · see citing works

Managing Fatigue in Patients With Interstitial Lung Disease

Kahlmann, V., Moor, C. C., & Wijsenbeek, M. S. (2020) · CHEST Journal · doi:10.1016/j.chest.2020.04.047 · Cited by 49 · see citing works

Comorbidity burden and survival in patients with idiopathic pulmonary fibrosis: the EMPIRE registry study

Jovanović, D. M., Šterclová, M., Moğulkoç, N., Lewandowska, K. B., Müller, V., Hájková, M., Studnicka, M., Tekavec-Trkanjec, J., Littnerová, S., Vašáková, M., et al. (2022) · Respiratory Research · doi:10.1186/s12931-022-02033-6 · Cited by 47 · see citing works

Updated Evaluation of the Safety, Efficacy and Tolerability of Pirfenidone in the Treatment of Idiopathic Pulmonary Fibrosis

Gulati, S., & Luckhardt, T. (2020) · Drug Healthcare and Patient Safety · doi:10.2147/dhps.s224007 · Cited by 46 · see citing works

Effectiveness and tolerability of antifibrotics in rheumatoid arthritis-associated interstitial lung disease

Juge, P., Hayashi, K., McDermott, G. C., Vanni, K. M. M., Kowalski, E. N., Qian, G., Bade, K. J., Saavedra, A. A., Dieudé, P., Dellaripa, P. F., et al. (2023) · Seminars in Arthritis and Rheumatism · doi:10.1016/j.semarthrit.2023.152312 · Cited by 43 · see citing works

Geriatric Nutritional Risk Index is a predictor of tolerability of antifibrotic therapy and mortality risk in patients with idiopathic pulmonary fibrosis

Mochizuka, Y., Suzuki, Y., Kono, M., Hasegawa, H., Hashimoto, D., Yokomura, K., Inoue, Y., Yasui, H., Hozumi, H., Karayama, M., et al. (2023) · Respirology · doi:10.1111/resp.14523 · Cited by 39 · see citing works

Comparative outcomes in patients receiving pirfenidone or nintedanib for idiopathic pulmonary fibrosis

Belhassen, M., Dalon, F., Nolin, M., & Van Ganse, E. (2021) · Respiratory Research · doi:10.1186/s12931-021-01714-y · Cited by 38 · see citing works

Antifibrotic Therapies and Progressive Fibrosing Interstitial Lung Disease (PF-ILD): Building on INBUILD

Shumar, J. N., Chandel, A., & King, C. S. (2021) · Journal of Clinical Medicine · doi:10.3390/jcm10112285 · Cited by 34 · see citing works

TGF-β inhibitors: the future for prevention and treatment of liver fibrosis?

Wang, W., Gao, Y., Chen, Y., Cheng, M., Sang, Y., Wei, L., Dai, R., Wang, Y., & Zhang, L. (2025) · Frontiers in Immunology · doi:10.3389/fimmu.2025.1583616 · Cited by 35 · see citing works

Patient expectations, experiences and satisfaction with nintedanib and pirfenidone in idiopathic pulmonary fibrosis: a quantitative study

Moor, C. C., Mostard, R. L. M., Grutters, J. C., Bresser, P., Aerts, J. G. J. V., Dirksen, C. D., Kimman, M., & Wijsenbeek, M. S. (2020) · Respiratory Research · doi:10.1186/s12931-020-01458-1 · Cited by 33 · see citing works

Efficacy, safety, and tolerability of combined pirfenidone and N-acetylcysteine therapy: a systematic review and meta-analysis

Shi, H., Yin, D., Bonella, F., Kreuter, M., Oltmanns, U., Li, X., Peng, S., & Wei, L. (2020) · BMC Pulmonary Medicine · doi:10.1186/s12890-020-1121-2 · Cited by 32 · see citing works

Advances in Targeted Therapy for Progressive Fibrosing Interstitial Lung Disease

Gibson, C. D., Kugler, M. C., Deshwal, H., Munger, J. S., & Condos, R. (2020) · Lung · doi:10.1007/s00408-020-00370-1 · Cited by 31 · see citing works

Pirfenidone in idiopathic pulmonary fibrosis: real-life experience in the referral centre of Siena

Vietri, L., Cameli, P., Perruzza, M., Cekorja, B., Bergantini, L., d’Alessandro, M., Refini, R. M., Pieroni, M., Fossi, A., Bennett, D., et al. (2020) · Therapeutic Advances in Respiratory Disease · doi:10.1177/1753466620906326 · Cited by 30 · see citing works

Pirfenidone in real life: A retrospective observational multicentre study in Italian patients with idiopathic pulmonary fibrosis

Vancheri, C., Sebastiani, A., Tomassetti, S., Pesci, A., Rogliani, P., Tavanti, L. M., Luppi, F., Harari, S. A., Rottoli, P., Ghirardini, A., et al. (2019) · Respiratory Medicine · doi:10.1016/j.rmed.2019.08.006 · Cited by 29 · see citing works

Guidelines of the Polish Respiratory Society for Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis

Piotrowski, W. J., Bestry, I., Białas, A. J., Boros, P. W., Grzanka, P., Jassem, E., Jastrzębski, D., Klimczak, D., Langfort, R., Lewandowska, K. B., et al. (2020) · Advances in respiratory medicine · doi:10.5603/arm.2020.0081 · Cited by 28 · see citing works

Pharmacological Interactions of Nintedanib and Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis in Times of COVID-19 Pandemic

López-Matencio, J. M. S., Gómez, M., Vicente‐Rabaneda, E. F., González-Gay, M. Á., Ancochea, J., & Castañeda, S. (2021) · Pharmaceuticals · doi:10.3390/ph14080819 · Cited by 28 · see citing works

The safety and tolerability of pirfenidone for bronchiolitis obliterans syndrome after hematopoietic cell transplant (STOP-BOS) trial

Matthaiou, E. I., Sharifi, H., O’Donnell, C. T., Chiu, W., Owyang, C. G., Chatterjee, P., Turk, I., Johnston, L., Brondstetter, T. I., Morris, K., et al. (2022) · Bone Marrow Transplantation · doi:10.1038/s41409-022-01716-4 · Cited by 28 · see citing works

Real-world safety profiles of pirfenidone and nintedanib in idiopathic pulmonary fibrosis patients

Fournier, D., Jouneau, S., Bouzillé, G., Polard, É., Osmont, M., & Scailteux, L. (2022) · Pulmonary Pharmacology & Therapeutics · doi:10.1016/j.pupt.2022.102149 · Cited by 28 · see citing works

Longitudinal clinical outcomes in a real-world population of patients with idiopathic pulmonary fibrosis: the PROOF registry

Wuyts, W. A., Dahlqvist, C., Slabbynck, H., Schlesser, M., Gusbin, N., Compère, C., Maddens, S., Lee, Y.-C., Kirchgaessler, K.-U., Bartley, K., et al. (2019) · Respiratory Research · doi:10.1186/s12931-019-1182-z · Cited by 28 · see citing works

Phase 2, Double-Blind, Placebo-controlled Trial of a c-Jun N-Terminal Kinase Inhibitor in Idiopathic Pulmonary Fibrosis

de Mattos, W. L. L. D., Khalil, N., Spencer, L. G., Bonella, F., Folz, R. J., Rolf, J. D., Moğulkoç, N., LANCASTER, L. H., Jenkins, G. R., Lynch, D. A., et al. (2024) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202310-1907oc · Cited by 26 · see citing works

Switching antifibrotics in patients with idiopathic pulmonary fibrosis: a multi-center retrospective cohort study

Suzuki, Y., Mori, K., Aono, Y., Kono, M., Hasegawa, H., Yokomura, K., Naoi, H., Hozumi, H., Karayama, M., Furuhashi, K., et al. (2021) · BMC Pulmonary Medicine · doi:10.1186/s12890-021-01587-3 · Cited by 25 · see citing works

The Effectiveness, Safety, and Tolerability of Pirfenidone in Idiopathic Pulmonary Fibrosis: A Retrospective Study

Hanta, İ., Çilli, A., & Sevinç, C. (2019) · Advances in Therapy · doi:10.1007/s12325-019-00928-3 · Cited by 25 · see citing works

Safety and tolerability of combination therapy with pirfenidone and nintedanib for idiopathic pulmonary fibrosis: A multicenter retrospective observational study in Japan

Hisata, S., Bando, M., Homma, S. A., Kataoka, K., Ogura, T., Izumi, S., Sakamoto, S., Watanabe, K., Saito, Y., Shimizu, Y., et al. (2021) · Respiratory Investigation · doi:10.1016/j.resinv.2021.04.005 · Cited by 24 · see citing works

Patient journey and treatment patterns in adults with IPF based on health care data in Sweden from 2001 to 2015

Sköld, C. M., Arnheim‐Dahlström, L., Bartley, K., Janson, C., Kirchgaessler, K.-U., LEVINE, A. M., & Ferrara, G. (2019) · Respiratory Medicine · doi:10.1016/j.rmed.2019.06.001 · Cited by 23 · see citing works

Recommandations pratiques pour le diagnostic et la prise en charge de la fibrose pulmonaire idiopathique – Actualisation 2021. Version courte

Cottin, V., Bonniaud, P., Cadranel, J. L., Crestani, B., Jouneau, S., Marchand-Adam, S., Nunès, H., Wémeau-Stervinou, L., Bergot, E., Blanchard, É., et al. (2022) · Revue des Maladies Respiratoires · doi:10.1016/j.rmr.2022.01.005 · Cited by 23 · see citing works

Pharmakotherapie der idiopathischen Lungenfibrose (ein Update) und anderer progredienter pulmonaler Fibrosen

Behr, J., Bonella, F., Frye, B. C., Günther, A., Hagmeyer, L., Henes, J. C., Klemm, P., Koschel, D., Kreuter, M., Leuschner, G., et al. (2023) · Pneumologie · doi:10.1055/a-1983-6796 · Cited by 21 · see citing works

Gastrointestinal pirfenidone adverse events in idiopathic pulmonary fibrosis depending on diet: the MADIET clinical trial

Molina‐Molina, M., Shull, J. G., Vicens‐Zygmunt, V., Rivera‐Ortega, P., Antoniou, K., Bonella, F., Renzoni, E. A., Russell, A., Maher, T. M., Vancheri, A., et al. (2023) · European Respiratory Journal · doi:10.1183/13993003.00262-2023 · Cited by 21 · see citing works

Cardiac fibrosis: emerging agents in preclinical and clinical development

Spoladore, R., Falasconi, G., Fiore, G., Maio, S. D., Preda, A., Slavich, M., Margonato, A., & Fragasso, G. (2020) · Expert Opinion on Investigational Drugs · doi:10.1080/13543784.2021.1868432 · Cited by 19 · see citing works

Improved Survival of IPF patients Treated With Antifibrotic Drugs Compared With Untreated Patients

Platenburg, M. G. J. P., van Moorsel, C. H. M., Wiertz, I. A., van der Vis, J. J., Vorselaars, A. D. M., Veltkamp, M., & Grutters, J. C. (2023) · Lung · doi:10.1007/s00408-023-00628-4 · Cited by 19 · see citing works

The tolerability and efficacy of antifibrotic therapy in patients with idiopathic pulmonary fibrosis: Results from a real-world study

Zhao, R., Xie, B., Wang, X., Zhang, X., Ren, Y., Wang, C., & Dai, H. (2024) · Pulmonary Pharmacology & Therapeutics · doi:10.1016/j.pupt.2024.102287 · Cited by 21 · see citing works

A 2-Year Observational Study in Patients Suffering from Idiopathic Pulmonary Fibrosis and Treated with Pirfenidone: A French Ancillary Study of PASSPORT

Jouneau, S., Gamez, A. S., Traclet, J., Nunès, H., Marchand-Adam, S., Kessler, R., Israël‐Biet, D., Borie, R., Strombom, I., Scalori, A., et al. (2019) · Respiration · doi:10.1159/000496735 · Cited by 19 · see citing works

A randomized phase 1 evaluation of deupirfenidone, a novel deuterium-containing drug candidate for interstitial lung disease and other inflammatory and fibrotic diseases

Chen, M. C., Korth, C. C., Harnett, M. D., Elenko, E., & Lickliter, J. D. (2022) · Clinical Pharmacology in Drug Development · doi:10.1002/cpdd.1040 · Cited by 18 · see citing works

A multicentre retrospective observational study on Polish experience of pirfenidone therapy in patients with idiopathic pulmonary fibrosis: the PolExPIR study

Majewski, S., Białas, A. J., Buchczyk, M., Gomółka, P., Górska, K., Jagielska-Len, H., Jarzemska, A., Jassem, E., Jastrzębski, D., Kania, A., et al. (2020) · BMC Pulmonary Medicine · doi:10.1186/s12890-020-1162-6 · Cited by 18 · see citing works

Antifibrotic drugs in connective tissue disease-related interstitial lung disease (CTD-ILD): from mechanistic insights to therapeutic applications

Erre, G. L., Sebastiani, M., Manfredi, A., Gerratana, E., Atzeni, F., Passiu, G., & Mangoni, A. A. (2021) · Drugs in Context · doi:10.7573/dic.2020-8-6 · Cited by 16 · see citing works

French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis – 2021 update. Full-length version

Cottin, V., Bonniaud, P., Cadranel, J. L., Crestani, B., Jouneau, S., Marchand-Adam, S., Nunès, H., Wémeau-Stervinou, L., Bergot, E., Blanchard, É., et al. (2022) · Respiratory Medicine and Research · doi:10.1016/j.resmer.2022.100948 · Cited by 17 · see citing works

COVID-19 and fibrosis: Mechanisms, clinical relevance, and future perspectives

Saifi, M. A., Bansod, S., & Godugu, C. (2022) · Drug Discovery Today · doi:10.1016/j.drudis.2022.103345 · Cited by 15 · see citing works

An updated safety review of the drug treatments for idiopathic pulmonary fibrosis

Sgalla, G., Comes, A., & Richeldi, L. (2021) · Expert Opinion on Drug Safety · doi:10.1080/14740338.2021.1921143 · Cited by 15 · see citing works

Efficacy and Safety of Pirfenidone in Advanced Versus Non-Advanced Idiopathic Pulmonary Fibrosis: Post-Hoc Analysis of Six Clinical Studies

Behr, J., Nathan, S. D., Costabel, U., Albera, C., Wuyts, W. A., Glassberg, M. K., Haller, H., Alvaro, G., Gilberg, F., Samara, K. D., et al. (2023) · Advances in Therapy · doi:10.1007/s12325-023-02565-3 · Cited by 14 · see citing works

A comprehensive and practical approach to the management of idiopathic pulmonary fibrosis

Moran‐Mendoza, O., Colman, R., Kalluri, M., Cabalteja, C., & Harle, I. A. (2019) · Expert Review of Respiratory Medicine · doi:10.1080/17476348.2019.1627204 · Cited by 15 · see citing works

Safety and tolerability of pirfenidone in asbestosis: a prospective multicenter study

Miedema, J., Moor, C. C., Veltkamp, M., Baart, S. J., Lie, N. S. L., Grutters, J. C., Wijsenbeek, M. S., & Mostard, R. L. M. (2022) · Respiratory Research · doi:10.1186/s12931-022-02061-2 · Cited by 14 · see citing works

Rationale for the Use of Pirfenidone in Heart Failure With Preserved Ejection Fraction

Graziani, F., Lillo, R., & Crea, F. (2021) · Frontiers in Cardiovascular Medicine · doi:10.3389/fcvm.2021.678530 · Cited by 13 · see citing works

Effectiveness of Pharmacist–Physician Collaborative Management for Patients With Idiopathic Pulmonary Fibrosis Receiving Pirfenidone

Satsuma, Y., Ikesue, H., Kusuda, K., Maeda, M., Muroi, N., Mori, R., Kogo, M., Hirabayashi, R., Nagata, K., Nakagawa, A., et al. (2020) · Frontiers in Pharmacology · doi:10.3389/fphar.2020.529654 · Cited by 11 · see citing works

Pirfenidone 5-hydroxylation is mainly catalysed by CYP1A2 and partly catalysed by CYP2C19 and CYP2D6 in the human liver

Zhang, Y., Sato, R., Fukami, T., Nakano, M., & Nakajima, M. (2021) · Xenobiotica · doi:10.1080/00498254.2021.2007553 · Cited by 11 · see citing works

Post-authorisation safety study of burosumab use in paediatric, adolescent and adult patients with X-linked hypophosphataemia: rationale and description

Brandi, M. L., Ariceta, G., Beck‐Nielsen, S. S., Boot, A. M., Briot, K., Collantes, C. D. L., Emma, F., Giannini, S., Haffner, D., Keen, R. W., et al. (2022) · Therapeutic Advances in Chronic Disease · doi:10.1177/20406223221117471 · Cited by 11 · see citing works

Factors associated with dose reduction of pirfenidone in patients with idiopathic pulmonary fibrosis: A study based on real-world clinical data

Kim, J., Chung, C., Cho, H. S., & Kim, H. C. (2023) · PLoS ONE · doi:10.1371/journal.pone.0281295 · Cited by 11 · see citing works

A roadmap to precision treatments for familial pulmonary fibrosis

Hurley, K., Ozaki, M., Philippot, Q., Galvin, L., Crosby, D., Kirwan, M., Gill, D. R., Alysandratos, K., Jenkins, G. R., Griese, M., et al. (2024) · EBioMedicine · doi:10.1016/j.ebiom.2024.105135 · Cited by 10 · see citing works

Diffuse Alveolar Hemorrhage Following Pirfenidone Initiation

Dull, S. K., Jagan, N., Moore, D. R., DePew, Z. S., Morrow, L. E., & Malesker, M. A. (2019) · Journal of Pharmacy Practice · doi:10.1177/0897190019841731 · Cited by 10 · see citing works

Recommandations pratiques pour le diagnostic et la prise en charge de la fibrose pulmonaire idiopathique – Actualisation 2021. Version intégrale

Cottin, V., Bonniaud, P., Cadranel, J. L., Crestani, B., Jouneau, S., Marchand-Adam, S., Nunès, H., Wémeau-Stervinou, L., Bergot, E., Blanchard, É., et al. (2022) · Revue des Maladies Respiratoires · doi:10.1016/j.rmr.2022.01.014 · Cited by 9 · see citing works

Pirfenidone in the kaleidoscope: reflecting mechanisms through different angles

Τzouvelekis, Α., & Wolters, P. J. (2018) · European Respiratory Journal · doi:10.1183/13993003.02046-2018 · Cited by 9 · see citing works

Pirfenidone for the treatment of bronchiolitis obliterans syndrome related to chronic graft-versus-host disease

Sharifi, H., Moss, C. T., Musa, Z., Bell, A., O’Donnell, C. T., Borges, C. H., Matthaiou, E. I., Johnston, L., Galbán, C. J., Sheshadri, A., et al. (2025) · Blood Advances · doi:10.1182/bloodadvances.2025016122 · Cited by 8 · see citing works

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Xiao, Y., Hoorain, I., Zhang, L., Bellusci, S., Jin, X., Yang, H., & Zhang, J.-S. (2025) · Frontiers in Immunology · doi:10.3389/fimmu.2025.1725041 · Cited by 8 · see citing works

Treatment of Idiopathic Pulmonary Fibrosis with Capsule or Tablet Formulations of Pirfenidone in the Real-Life French RaDiCo-ILD Cohort

Cottin, V., Guéguen, S., Nunès, H., Jouneau, S., Crestani, B., Bonniaud, P., Wémeau-Stervinou, L., Israël‐Biet, D., Reynaud‐Gaubert, M., Gondouin, A., et al. (2021) · Advances in Therapy · doi:10.1007/s12325-021-01961-x · Cited by 8 · see citing works

Healthcare resource use and associated costs in patients receiving pirfenidone or nintedanib for idiopathic pulmonary fibrosis

Cottin, V., Spagnolo, P., Bonniaud, P., Dalon, F., Nolin, M., Kirchgässler, K.-U., Ganse, E. H. V., & Belhassen, M. (2022) · Respiratory Medicine and Research · doi:10.1016/j.resmer.2022.100951 · Cited by 8 · see citing works

Gender in idiopathic pulmonary fibrosis diagnosis: time to address unconscious bias

Strek, M. E. (2020) · Thorax · doi:10.1136/thoraxjnl-2020-214569 · Cited by 8 · see citing works

Post-marketing safety concerns with pirfenidone and nintedanib: an analysis of individual case safety reports from the FDA adverse event reporting system database and the Japanese adverse drug event report databases

Wang, T., Cui, Z., Ou, Y., Lou, S., Chen, H., Zhu, C., Zhou, L., & Zou, F. (2025) · Frontiers in Pharmacology · doi:10.3389/fphar.2025.1530697 · Cited by 7 · see citing works

Effects of the COVID-19 pandemic on the follow-up and treatment of patients with idiopathic pulmonary fibrosis: a cross-sectional, multicentre phone call survey

Çoşkun, F., Hanta, I., Ĉilli, A., Ozkaya, G., Ursavaş, A., & Sevinç, C. (2021) · BMJ Open · doi:10.1136/bmjopen-2021-050578 · Cited by 4 · see citing works

Exhaled Biomarkers in Idiopathic Pulmonary Fibrosis—A Six-Month Follow-up Study in Patients Treated with Pirfenidone

Jaśkiewicz, K., Mycroft, K., Maskey‐Warzęchowska, M., Paralusz-Stec, K., Siemiez, N., Nejman‐Gryz, P., Barnaś, M., Krenke, R., & Górska, K. (2020) · Journal of Clinical Medicine · doi:10.3390/jcm9082523 · Cited by 7 · see citing works

Evaluating fatigue and excessive daytime sleepiness: a comparative analysis of prevalence and correlating factors in interstitial lung disease patients and healthy controls

Ouyang, X., Shen, Q., Zhou, S., Zhou, P., Song, M. S., Guo, T., Guo, W., Zhang, Y., & Peng, H. (2024) · Annals of Medicine · doi:10.1080/07853890.2024.2398729 · Cited by 6 · see citing works

MUC5B Polymorphism in Patients with Idiopathic Pulmonary Fibrosis—Does It Really Matter?

Lewandowska, K. B., Lechowicz, U., Roży, A., Falis, M., Błasińska, K., Jakubowska, L., Franczuk, M., Żołnowska, B., Gryczka-Wróbel, J., Radwan-Röhrenschef, P., et al. (2025) · International Journal of Molecular Sciences · doi:10.3390/ijms26052218 · Cited by 5 · see citing works

Effectiveness and safety of pirfenidone for radiation-induced lung injury in non-small cell lung cancer: a retrospective pilot study

Liu, X., Lu, F., Yang, H., Liao, G., Zhao, L., Zou, D., He, T., Hu, Y., Wang, L., Xia, J., et al. (2025) · BMC Cancer · doi:10.1186/s12885-025-14896-1 · Cited by 5 · see citing works

Pharmacological Treatment of Idiopathic Pulmonary Fibrosis (Update) and Progressive Pulmonary Fibroses: S2k Guideline of the German Respiratory Society

Behr, J., Bonella, F., Frye, B. C., Günther, A., Hagmeyer, L., Henes, J., Klemm, P., Koschel, D., Kreuter, M., Leuschner, G., et al. (2024) · Respiration · doi:10.1159/000540856 · Cited by 5 · see citing works

Persistence of Antifibrotic Therapy in Patients with Idiopathic Pulmonary Fibrosis: A Pulmonary Fibrosis Foundation Patient Registry Study

Kulkarni, T., Flaherty, K. R., Gupta, S., Tu, Y.-H., & Case, A. H. (2024) · Annals of the American Thoracic Society · doi:10.1513/annalsats.202404-430rl · Cited by 5 · see citing works

Clinical course of mild-to-moderate idiopathic pulmonary fibrosis during therapy with pirfenidone: Results of the non-interventional study AERplus

Schreiber, J., Schütte, W., Koerber, W., Seese, B., Koschel, D., Neuland, K., & Grohé, C. (2024) · Pneumologie · doi:10.1055/a-2267-2074 · Cited by 5 · see citing works

Stevens-Johnson syndrome/toxic epidermal necrolysis induced by pirfenidone

de Perosanz-Lobo, D., Fernández‐Nieto, D., Burgos‐Blasco, P., Aroca-Ruiz, M., & Fernández-Guarino, M. (2021) · Indian Journal of Dermatology Venereology and Leprology · doi:10.25259/ijdvl_61_20 · Cited by 5 · see citing works

A Narrative Review of Salivary Gland Extracellular Matrix and Sjögren’s Syndrome: Research Status and Future Prospects

Su, H., Fan, X., Nie, C., Tang, X., Hu, J., Xu, K., Zhang, L., & Ma, D. (2026) · Biomolecules · doi:10.3390/biom16010072 · Cited by 6 · see citing works

Analysis of real-world data and a mouse model indicates that pirfenidone causes pellagra

Kuronuma, K., Susai, N., Kuroita, T., Yamamoto, H., Yoshioka, T., Kaneko, S., & Chiba, H. (2022) · ERJ Open Research · doi:10.1183/23120541.00245-2022 · Cited by 4 · see citing works

Improvement of psoriatic skin lesions following pirfenidone use in patients with fibrotic lung disease

Karampitsakos, Τ., Sotiropoulou, V., Katsaras, M., & Tzouvelekis, A. (2023) · BMJ Case Reports · doi:10.1136/bcr-2022-252591 · Cited by 4 · see citing works

Real-world patterns of pirfenidone use and safety in patients with idiopathic pulmonary fibrosis in Canada: Data from INSPIRATION PLUS

Ryerson, C. J., Kolb, M. R. J., Cox, G., Shapera, S., Swigris, J. J., Fell, C. D., O’Brien, M., Xue, C., Rassi, J., & Moran‐Mendoza, O. (2019) · Canadian Journal of Respiratory Critical Care and Sleep Medicine · doi:10.1080/24745332.2019.1586496 · Cited by 4 · see citing works

Deupirfenidone Compared with Pirfenidone and Placebo in Idiopathic Pulmonary Fibrosis (ELEVATE-IPF): a Phase 2b Randomized Placebo-Controlled Trial

Maher, T. M., Hamblin, M. J., Choi, W.-I., Case, A. H., Tomos, I. P., Tzouvelekis, A. E., Shore, J. E., Bergna, M. A., Golod, D., Elenko, E., et al. (2026) · American Journal of Respiratory and Critical Care Medicine · doi:10.1093/ajrccm/aamag155 · Cited by 4 · see citing works

Advances in Heart Failure with Preserved Ejection Fraction Management - The Role of Sacubitril-Valsartan, Pirfenidone, Spironolactone and Empagliflozin: Is Success a Series of Small Victories?

Γιαννόπουλος, Γ., Kousta, M. S., Anagnostopoulos, I., Karageorgiou, S., Myrovali, E., Deftereos, G., Fragakis, N., Siasos, G., & Vassilikos, V. P. (2023) · Current Pharmaceutical Design · doi:10.2174/1381612829666230202141437 · Cited by 3 · see citing works

Anti-Radiofibrosis Effect of Dicliptera chinensis Polysaccharide on Rat Dermal Fibroblasts Via The TGF-β1/Smads/CTGF Signaling Pathway

Huang, Y., Zhao, L., Zhao, Y., Fan, Y., Gao, L., Lu, H., Wang, X., Mo, D., & Wang, D. (2024) · International Dental Journal · doi:10.1016/j.identj.2024.09.024 · Cited by 3 · see citing works

Idiopathic pulmonary fibrosis: accurate diagnosis and early treatment

Morais, A. (2019) · Jornal Brasileiro de Pneumologia · doi:10.1590/1806-3713/e20190353 · Cited by 3 · see citing works

Predictive factors for the long-term use of pirfenidone in patients with fibrosing interstitial lung disease

Katano, T., Sekine, A., Ikeda, S., Yamakawa, H., Misumi, T., Okabayashi, H., Okuda, R., Kitamura, H., Baba, T., Komatsu, S., et al. (2021) · Respiratory Investigation · doi:10.1016/j.resinv.2021.01.002 · Cited by 3 · see citing works

Clinical Insights and Therapeutic Strategies for the Treatment of Interstitial Lung Disease in Patients with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: Current Trends and Future Directions

Fijołek, J., & Śniady, A. (2025) · Journal of Clinical Medicine · doi:10.3390/jcm14134631 · Cited by 3 · see citing works

Pirfenidone treatment attenuates fibrosis in autosomal dominant polycystic kidney disease

Remadevi, V., Jamadar, A., Varghese, M. M., Yang, H., Gunewardena, S., Wallace, D. P., & Rao, R. (2026) · JCI Insight · doi:10.1172/jci.insight.207552 · Cited by 2 · see citing works

Validated UV-Spectrophotometric Method for Pirfenidone Estimation in Bulk Drug, Marketed Tablet, and Nanoparticle Formulation

Dudhat, K. R., Goswami, J., & Patel, H. (2024) · Pharmaceutical Chemistry Journal · doi:10.1007/s11094-024-03196-w · Cited by 2 · see citing works

Geriatric nutrition risk index as an indicator of acute exacerbation and mortality risk in patients with idiopathic pulmonary fibrosis

Deniz, P. P., Hanta, İ., Çetinkaya, P. D., & Küçükyalçın, S. (2025) · BMC Geriatrics · doi:10.1186/s12877-025-06377-8 · Cited by 2 · see citing works

The cardiovascular system in idiopathic pulmonary fibrosis

Chong, S. G., Yanagihara, T., & Kolb, M. R. J. (2020) · European Respiratory Society eBooks · doi:10.1183/2312508x.10028319 · Cited by 2 · see citing works

Interstitial Lung Disease and ANCA-Associated Vasculitis

Flores-Suárez, L. F., & Sacoto, G. (2019) · Current Treatment Options in Rheumatology · doi:10.1007/s40674-019-00127-9 · Cited by 2 · see citing works

Pulmonary Fibrosis

Rezaee, R., & Hosseinzadeh, H. (2021) · Elsevier eBooks · doi:10.1016/b978-0-12-820472-6.00131-6 · Cited by 2 · see citing works

Double Lung Transplantation for Idiopathic Pulmonary Fibrosis in a Patient with a History of Liver Transplantation and Prolonged Journey for Disease-Specific Antifibrotic Therapy

Majewski, S., Królikowska, M., Costabel, U., Piotrowski, W. J., & Ochman, M. (2022) · Case Reports in Pulmonology · doi:10.1155/2022/4054339 · Cited by 2 · see citing works

Editorial: Pulmonary fibrosis and lung carcinogenesis: do myofibroblasts and cancer-associated fibroblasts share a common identity?

de Totero, D., Barisione, E., & Clini, E. M. (2024) · Frontiers in Oncology · doi:10.3389/fonc.2024.1389532 · Cited by 2 · see citing works

Safety, effectiveness, and usefulness of higher-dose tablets of generic pirfenidone in patients with IPF: a nationwide observational study in South Korea

Kang, J., Lee, K. H., Lee, J. H., Jeong, Y. Y., Choi, S. M., Kim, H. C., Park, J. H., Lee, H.-K., Yong, S. J., Choi, H. S., et al. (2024) · Frontiers in Pharmacology · doi:10.3389/fphar.2024.1451447 · Cited by 1 · see citing works

Real-World Comprehensive Disease Management of Patients With Idiopathic Pulmonary Fibrosis

Mason, W. R., McLaughlin, S., Dedopoulos, S., Mahoney, E., Meadows, T., Stauffer, J. L., & LANCASTER, L. H. (2019) · Current Respiratory Medicine Reviews · doi:10.2174/1573398x15666190212155051 · Cited by 1 · see citing works

Idiopathic interstitial pneumonias

Averyanov, A., Коган, Е. А., Lesnyak, V., Stepanyan, I. E., & Васильевна, Д. О. (2019) · Elsevier eBooks · doi:10.1016/b978-0-12-815375-8.00002-9 · Cited by 1 · see citing works

Comparative Outcomes in Patients Receiving Pirfenidone or Nintedanib for Idiopathic Pulmonary FibrosisPreprint

Belhassen, M., Dalon, F., Nolin, M., & Ganse, E. H. V. (2020) · Research Square · doi:10.21203/rs.3.rs-124214/v1 · Cited by 1 · see citing works

Safety and Efficacy of Early and Long-Term Use of Pirfenidone in Idiopathic Pulmonary Fibrosis

Tzilas, V., Tzouvelekis, A., & Bouros, D. E. (2019) · Respiration · doi:10.1159/000497391

Accompagnement par une infirmière spécialisée des patients recevant un traitement anti-fibrosant pulmonaire

Guillemot, A., Nasser, M., Paganon, C., Ahmad, K., Traclet, J., & Cottin, V. (2020) · Revue des Maladies Respiratoires Actualités · doi:10.1016/j.rmra.2019.11.522

Antifibrotische Therapie der idiopathischen pulmonalen Fibrose

Markart, P., Drakopanagiotakis, F., & Wygrecka, M. (2020) · Der Pneumologe · doi:10.1007/s10405-019-00296-3

Late Breaking Abstract - Long-term safety of pirfenidone in a real-world setting: final results from the prospective, observational PASSPORT registry

Cottin, V., Koschel, D., Günther, A., Albera, C., Azuma, A., Sköld, M. C., Tomassetti, S., Hormel, P., Stauffer, J. L., Strombom, I., et al. (2017) · doi:10.1183/1393003.congress-2017.pa2806 · Cited by 1 · see citing works

Pirfenidone post-authorization safety registry (PASSPORT) – Interim analysis of IPF treatment

Koschel, D., Cottin, V., Sköld, M. C., Tomassetti, S., Azuma, A., Giot, C., Yocum, D. E., Hamza, S., & Maher, T. M. (2014) · European Respiratory Journal · doi:10.1183/13993003/erj.44.suppl_58.1904 · Cited by 15 · see citing works

S11 Pirfenidone Post-authorisation Safety Registry (passport)-interim Analysis Of Ipf Treatment

Maher, T. M., Cottin, V., Skoeld, M., Tomassetti, S., Azuma, A., Giot, C., Yocum, D. E., Hamza, S., & Koschel, D. (2014) · Thorax · doi:10.1136/thoraxjnl-2014-206260.17 · Cited by 6 · see citing works

P12 Pirfenidone post-authorisation safety registry (PASSPORT) – update and concomitant use of N-acetylcysteine and/or corticosteroids

Maher, T. M., Cottin, V., Azuma, A., Groves, L., Hormel, P., Sköld, M. C., Tomassetti, S., & Koschel, D. (2015) · Thorax · doi:10.1136/thoraxjnl-2015-207770.149

Pirfenidone post-authorization safety registry (PASSPORT) update

Koschel, D., Cottin, V., Maher, T. M., Azuma, A., Groves, L., Hormel, P., Sköld, M. C., & Tomassetti, S. (2016) · Pneumologie · doi:10.1055/s-0036-1572065 · Cited by 3 · see citing works