Disease progression in idiopathic pulmonary fibrosis under anti-fibrotic treatment
Main Article Content
Keywords
IPF, disease progression, nintedanib, pirfenidone
Abstract
Idiopathic pulmonary fibrosis (IPF) is the most common progressive interstitial disease of unknown etiology. The course of disease is not possible to predict. Frequent monitoring using multiple assessments is important to evaluate disease progression. Currently, there is no consensus on how progression should be defined. Nintedanib and pirfenidone slow the progression of IPF, but the disease can progress even under anti-fibrotic treatment. The goal of this review is to examine and summarize the current data about IPF progression in patients who were on anti-fibrotic treatment. Also, we outline the limitations of the tests used for disease progression.
References
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15. Richeldi L, Costabel U, Selman M, et al. Efficacy of a Tyrosine Kinase Inhibitor in Idiopathic Pulmonary Fibrosis. N Engl J Med. 2011;365(12):1079–1087. doi: 10.1056/NEJMoa1103690.
16. Costabel U, Inoue Y, Richeldi L, et al. Efficacy of Nintedanib in Idiopathic Pulmonary Fibrosis across Prespecified Subgroups in INPULSIS. Am J Respir Crit Care Med. 2016;193(2):178–185. doi: 10.1164/rccm.201503-0562OC.
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18. Conte E, Gili E, Fagone E, Fruciano M, Iemmolo M, Vancheri C. Effect of pirfenidone on proliferation, TGF-β-induced myofibroblast differentiation and fibrogenic activity of primary human lung fibroblasts. Eur J Pharm Sci Off J Eur Fed Pharm Sci. 2014;58:13–19. doi: 10.1016/j.ejps.2014.02.014.
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21. Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet Lond Engl. 2011;377(9779):1760–1769. doi: 10.1016/S0140-6736(11)60405-4.
22. King TE, Bradford WZ, Castro-Bernardini S, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083–2092. doi: 10.1056/NEJMoa1402582.
23. Zurkova M, Kriegova E, Kolek V, et al. Effect of pirfenidone on lung function decline and survival: 5-yr experience from a real-life IPF cohort from the Czech EMPIRE registry. Respir Res. 2019;20(1):16. doi: 10.1186/s12931-019-0977-2.
24. Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. American Thoracic Society - AJRCCM; 2022;205(9):e18–e47. doi: 10.1164/rccm.202202-0399ST.
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26. Oltmanns U, Kahn N, Palmowski K, et al. Pirfenidone in idiopathic pulmonary fibrosis: real-life experience from a German tertiary referral center for interstitial lung diseases. Respir Int Rev Thorac Dis. 2014;88(3):199–207. doi: 10.1159/000363064.
27. Feng H, Zhao Y, Li Z, Kang J. Real-life experiences in a single center: efficacy of pirfenidone in idiopathic pulmonary fibrosis and fibrotic idiopathic non-specific interstitial pneumonia patients. Ther Adv Respir Dis. 2020;14:1753466620963015. doi: 10.1177/1753466620963015.
28. Vianello A, Salton F, Molena B, et al. Nintedanib Treatment for Idiopathic Pulmonary Fibrosis Patients Who Have Been Switched from Pirfenidone Therapy: A Retrospective Case Series Study. J Clin Med. 2020;9(2). doi: 10.3390/jcm9020422.
29. Suzuki Y, Mori K, Aono Y, et al. Switching antifibrotics in patients with idiopathic pulmonary fibrosis: a multi-center retrospective cohort study. BMC Pulm Med. 2021;21(1):221. doi: 10.1186/s12890-021-01587-3.
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31. Bando M, Yamauchi H, Ogura T, et al. Clinical Experience of the Long-term Use of Pirfenidone for Idiopathic Pulmonary Fibrosis. Intern Med Tokyo Jpn. 2016;55(5):443–448. doi: 10.2169/internalmedicine.55.5272.
32. Cerri S, Monari M, Guerrieri A, et al. Real-life comparison of pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis: A 24-month assessment. Respir Med. 2019;159:105803. doi: 10.1016/j.rmed.2019.105803.
33. Vietri L, Cameli P, Perruzza M, et al. Pirfenidone in idiopathic pulmonary fibrosis: real-life experience in the referral centre of Siena. Ther Adv Respir Dis. 2020;14:1753466620906326. doi: 10.1177/1753466620906326.
34. Behr J. Disease Progression in Idiopathic Pulmonary Fibrosis. FVC Is Not Enough. Am J Respir Crit Care Med. 2017;196(9):1094–1095. doi: 10.1164/rccm.201706-1246ED.
35. Nathan SD, Wanger J, Zibrak JD, Wencel ML, Burg C, Stauffer JL. Using forced vital capacity (FVC) in the clinic to monitor patients with idiopathic pulmonary fibrosis (IPF): pros and cons. Expert Rev Respir Med. 2021;15(2):175–181. doi: 10.1080/17476348.2020.1816831.
36. Antoniou KM, Walsh SL, Hansell DM, et al. Smoking-related emphysema is associated with idiopathic pulmonary fibrosis and rheumatoid lung. Respirol Carlton Vic. 2013;18(8):1191–1196. doi: 10.1111/resp.12154.
37. Pellegrino R, Viegi G, Brusasco V, et al. Interpretative strategies for lung function tests. Eur Respir J. European Respiratory Society; 2005;26(5):948–968. doi: 10.1183/09031936.05.00035205.
38. Hegewald MJ. Diffusing capacity. Clin Rev Allergy Immunol. 2009;37(3):159–166. doi: 10.1007/s12016-009-8125-2.
39. Sansores RH, Abboud RT, Kennell C, Haynes N. The effect of menstruation on the pulmonary carbon monoxide diffusing capacity. Am J Respir Crit Care Med. 1995;152(1):381–384. doi: 10.1164/ajrccm.152.1.7599851.
40. Kono K, Nishida Y, Moriyama Y, Yabe H, Taoka M, Sato T. Investigation of factors affecting the six-minute walk test results in hemodialysis patients. Ther Apher Dial Off Peer-Rev J Int Soc Apher Jpn Soc Apher Jpn Soc Dial Ther. 2014;18(6):623–627. doi: 10.1111/1744-9987.12177.
41. Sciurba F, Criner GJ, Lee SM, et al. Six-minute walk distance in chronic obstructive pulmonary disease: reproducibility and effect of walking course layout and length. Am J Respir Crit Care Med. 2003;167(11):1522–1527. doi: 10.1164/rccm.200203-166OC.
42. Kim GHJ, Weigt SS, Belperio JA, et al. Prediction of idiopathic pulmonary fibrosis progression using early quantitative changes on CT imaging for a short term of clinical 18–24-month follow-ups. Eur Radiol. 2020;30(2):726–734. doi: 10.1007/s00330-019-06402-6.
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44. Humphries SM, Yagihashi K, Huckleberry J, et al. Idiopathic Pulmonary Fibrosis: Data-driven Textural Analysis of Extent of Fibrosis at Baseline and 15-Month Follow-up. Radiology. 2017;285(1):270–278. doi: 10.1148/radiol.2017161177.
45. Sun H, Liu M, Kang H, et al. Idiopathic pulmonary fibrosis disease progression: a dynamic quantitative chest computed tomography follow-up analysis. Quant Imaging Med Surg. 2023;13(3):1488–1498. doi: 10.21037/qims-22-843.
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2. Nalysnyk L, Cid-Ruzafa J, Rotella P, Esser D. Incidence and prevalence of idiopathic pulmonary fibrosis: review of the literature. Eur Respir Rev Off J Eur Respir Soc. 2012;21(126):355–361. doi: 10.1183/09059180.00002512.
3. Hutchinson J, Fogarty A, Hubbard R, McKeever T. Global incidence and mortality of idiopathic pulmonary fibrosis: a systematic review. Eur Respir J. 2015;46(3):795–806. doi: 10.1183/09031936.00185114.
4. Raghu G, Collard HR, Egan JJ, et al. An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management. Am J Respir Crit Care Med. 2011;183(6):788–824. doi: 10.1164/rccm.2009-040GL.
5. Ley B, Collard HR, King TE. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183(4):431–440. doi: 10.1164/rccm.201006-0894CI.
6. Jo HE, Glaspole I, Moodley Y, et al. Disease progression in idiopathic pulmonary fibrosis with mild physiological impairment: analysis from the Australian IPF registry. BMC Pulm Med. 2018;18(1):19. doi: 10.1186/s12890-018-0575-y.
7. Raghu G. Idiopathic pulmonary fibrosis: lessons from clinical trials over the past 25 years. Eur Respir J. 2017;50(4):1701209. doi: 10.1183/13993003.01209-2017.
8. du Bois RM, Nathan SD, Richeldi L, Schwarz MI, Noble PW. Idiopathic pulmonary fibrosis: lung function is a clinically meaningful endpoint for phase III trials. Am J Respir Crit Care Med. 2012;186(8):712–715. doi: 10.1164/rccm.201206-1010PP.
9. Ley B, Ryerson CJ, Vittinghoff E, et al. A Multidimensional Index and Staging System for Idiopathic Pulmonary Fibrosis | Annals of Internal Medicine. 2022;156(10):684–691. doi: https://doi.org/10.7326/0003-4819-156-10-201205150-00004.
10. Schwartz DA, Van Fossen DS, Davis CS, et al. Determinants of progression in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1994;149(2 Pt 1):444–449. doi: 10.1164/ajrccm.149.2.8306043.
11. Rudd RM, Haslam PL, Turner-Warwick M. Cryptogenic fibrosing alveolitis. Relationships of pulmonary physiology and bronchoalveolar lavage to response to treatment and prognosis. Am Rev Respir Dis. 1981;124(1):1–8. doi: 10.1164/arrd.1981.124.1.1.
12. Turner-Warwick M, Haslam PL. The value of serial bronchoalveolar lavages in assessing the clinical progress of patients with cryptogenic fibrosing alveolitis. Am Rev Respir Dis. 1987;135(1):26–34. doi: 10.1164/arrd.1987.135.1.26.
13. Watters LC, Schwarz MI, Cherniack RM, et al. Idiopathic pulmonary fibrosis. Pretreatment bronchoalveolar lavage cellular constituents and their relationships with lung histopathology and clinical response to therapy. Am Rev Respir Dis. 1987;135(3):696–704. doi: 10.1164/arrd.1987.135.3.696.
14. Richeldi L, du Bois RM, Raghu G, et al. Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis. N Engl J Med. 2014;370(22):2071–2082. doi: 10.1056/NEJMoa1402584.
15. Richeldi L, Costabel U, Selman M, et al. Efficacy of a Tyrosine Kinase Inhibitor in Idiopathic Pulmonary Fibrosis. N Engl J Med. 2011;365(12):1079–1087. doi: 10.1056/NEJMoa1103690.
16. Costabel U, Inoue Y, Richeldi L, et al. Efficacy of Nintedanib in Idiopathic Pulmonary Fibrosis across Prespecified Subgroups in INPULSIS. Am J Respir Crit Care Med. 2016;193(2):178–185. doi: 10.1164/rccm.201503-0562OC.
17. Crestani B, Huggins JT, Kaye M, et al. Long-term safety and tolerability of nintedanib in patients with idiopathic pulmonary fibrosis: results from the open-label extension study, INPULSIS-ON. Lancet Respir Med. Elsevier; 2019;7(1):60–68. doi: 10.1016/S2213-2600(18)30339-4.
18. Conte E, Gili E, Fagone E, Fruciano M, Iemmolo M, Vancheri C. Effect of pirfenidone on proliferation, TGF-β-induced myofibroblast differentiation and fibrogenic activity of primary human lung fibroblasts. Eur J Pharm Sci Off J Eur Fed Pharm Sci. 2014;58:13–19. doi: 10.1016/j.ejps.2014.02.014.
19. Raghu G, Johnson WC, Lockhart D, Mageto Y. Mageto Y. Treatment of idiopathic pulmonary fibrosis with a new antifibrotic agent, Pirfenidone: results of a prospective, open-label Phase II study. Am J Resp Crit Care Med. 1999;159(4 Pt 1):1061-9. doi: 10.1164/ajrccm.159.4.9805017.
20. Nagai S, Hamada K, Shigematsu M, Taniyama M, Yamauchi S, Izumi T. Open-label compassionate use one year-treatment with pirfenidone to patients with chronic pulmonary fibrosis. Intern Med Tokyo Jpn. 2002;41(12):1118–1123. doi: 10.2169/internalmedicine.41.1118.
21. Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet Lond Engl. 2011;377(9779):1760–1769. doi: 10.1016/S0140-6736(11)60405-4.
22. King TE, Bradford WZ, Castro-Bernardini S, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083–2092. doi: 10.1056/NEJMoa1402582.
23. Zurkova M, Kriegova E, Kolek V, et al. Effect of pirfenidone on lung function decline and survival: 5-yr experience from a real-life IPF cohort from the Czech EMPIRE registry. Respir Res. 2019;20(1):16. doi: 10.1186/s12931-019-0977-2.
24. Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. American Thoracic Society - AJRCCM; 2022;205(9):e18–e47. doi: 10.1164/rccm.202202-0399ST.
25. Antoniou K, Markopoulou K, Tzouvelekis A, et al. Efficacy and safety of nintedanib in a Greek multicentre idiopathic pulmonary fibrosis registry: a retrospective, observational, cohort study. ERJ Open Res. European Respiratory Society; 2020;6(1). doi: 10.1183/23120541.00172-2019.
26. Oltmanns U, Kahn N, Palmowski K, et al. Pirfenidone in idiopathic pulmonary fibrosis: real-life experience from a German tertiary referral center for interstitial lung diseases. Respir Int Rev Thorac Dis. 2014;88(3):199–207. doi: 10.1159/000363064.
27. Feng H, Zhao Y, Li Z, Kang J. Real-life experiences in a single center: efficacy of pirfenidone in idiopathic pulmonary fibrosis and fibrotic idiopathic non-specific interstitial pneumonia patients. Ther Adv Respir Dis. 2020;14:1753466620963015. doi: 10.1177/1753466620963015.
28. Vianello A, Salton F, Molena B, et al. Nintedanib Treatment for Idiopathic Pulmonary Fibrosis Patients Who Have Been Switched from Pirfenidone Therapy: A Retrospective Case Series Study. J Clin Med. 2020;9(2). doi: 10.3390/jcm9020422.
29. Suzuki Y, Mori K, Aono Y, et al. Switching antifibrotics in patients with idiopathic pulmonary fibrosis: a multi-center retrospective cohort study. BMC Pulm Med. 2021;21(1):221. doi: 10.1186/s12890-021-01587-3.
30. Milger K, Kneidinger N, Neurohr C, Reichenberger F, Behr J. Switching to nintedanib after discontinuation of pirfenidone due to adverse events in IPF. Eur Respir J. 2015;46(4):1217–1221. doi: 10.1183/13993003.00584-2015.
31. Bando M, Yamauchi H, Ogura T, et al. Clinical Experience of the Long-term Use of Pirfenidone for Idiopathic Pulmonary Fibrosis. Intern Med Tokyo Jpn. 2016;55(5):443–448. doi: 10.2169/internalmedicine.55.5272.
32. Cerri S, Monari M, Guerrieri A, et al. Real-life comparison of pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis: A 24-month assessment. Respir Med. 2019;159:105803. doi: 10.1016/j.rmed.2019.105803.
33. Vietri L, Cameli P, Perruzza M, et al. Pirfenidone in idiopathic pulmonary fibrosis: real-life experience in the referral centre of Siena. Ther Adv Respir Dis. 2020;14:1753466620906326. doi: 10.1177/1753466620906326.
34. Behr J. Disease Progression in Idiopathic Pulmonary Fibrosis. FVC Is Not Enough. Am J Respir Crit Care Med. 2017;196(9):1094–1095. doi: 10.1164/rccm.201706-1246ED.
35. Nathan SD, Wanger J, Zibrak JD, Wencel ML, Burg C, Stauffer JL. Using forced vital capacity (FVC) in the clinic to monitor patients with idiopathic pulmonary fibrosis (IPF): pros and cons. Expert Rev Respir Med. 2021;15(2):175–181. doi: 10.1080/17476348.2020.1816831.
36. Antoniou KM, Walsh SL, Hansell DM, et al. Smoking-related emphysema is associated with idiopathic pulmonary fibrosis and rheumatoid lung. Respirol Carlton Vic. 2013;18(8):1191–1196. doi: 10.1111/resp.12154.
37. Pellegrino R, Viegi G, Brusasco V, et al. Interpretative strategies for lung function tests. Eur Respir J. European Respiratory Society; 2005;26(5):948–968. doi: 10.1183/09031936.05.00035205.
38. Hegewald MJ. Diffusing capacity. Clin Rev Allergy Immunol. 2009;37(3):159–166. doi: 10.1007/s12016-009-8125-2.
39. Sansores RH, Abboud RT, Kennell C, Haynes N. The effect of menstruation on the pulmonary carbon monoxide diffusing capacity. Am J Respir Crit Care Med. 1995;152(1):381–384. doi: 10.1164/ajrccm.152.1.7599851.
40. Kono K, Nishida Y, Moriyama Y, Yabe H, Taoka M, Sato T. Investigation of factors affecting the six-minute walk test results in hemodialysis patients. Ther Apher Dial Off Peer-Rev J Int Soc Apher Jpn Soc Apher Jpn Soc Dial Ther. 2014;18(6):623–627. doi: 10.1111/1744-9987.12177.
41. Sciurba F, Criner GJ, Lee SM, et al. Six-minute walk distance in chronic obstructive pulmonary disease: reproducibility and effect of walking course layout and length. Am J Respir Crit Care Med. 2003;167(11):1522–1527. doi: 10.1164/rccm.200203-166OC.
42. Kim GHJ, Weigt SS, Belperio JA, et al. Prediction of idiopathic pulmonary fibrosis progression using early quantitative changes on CT imaging for a short term of clinical 18–24-month follow-ups. Eur Radiol. 2020;30(2):726–734. doi: 10.1007/s00330-019-06402-6.
43. Egashira R, Raghu G. Quantitative computed tomography of the chest for fibrotic lung diseases: Prime time for its use in routine clinical practice? Respirol Carlton Vic. 2022;27(12):1008–1011. doi: 10.1111/resp.14351.
44. Humphries SM, Yagihashi K, Huckleberry J, et al. Idiopathic Pulmonary Fibrosis: Data-driven Textural Analysis of Extent of Fibrosis at Baseline and 15-Month Follow-up. Radiology. 2017;285(1):270–278. doi: 10.1148/radiol.2017161177.
45. Sun H, Liu M, Kang H, et al. Idiopathic pulmonary fibrosis disease progression: a dynamic quantitative chest computed tomography follow-up analysis. Quant Imaging Med Surg. 2023;13(3):1488–1498. doi: 10.21037/qims-22-843.
46. Collard HR, Moore BB, Flaherty KR, et al. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2007;176(7):636–643. doi: 10.1164/rccm.200703-463PP.
47. Collard HR, Ryerson CJ, Corte TJ, et al. Acute Exacerbation of Idiopathic Pulmonary Fibrosis. An International Working Group Report. Am J Respir Crit Care Med. 2016;194(3):265–275. doi: 10.1164/rccm.201604-0801CI.
48. Cilli A, Uzer F, Sevinç C, et al. Tolerability and efficacy of second-line antifibrotics in patients with idiopathic pulmonary fibrosis. Pulm Pharmacol Ther. 2021;71:102099. doi: 10.1016/j.pupt.2021.102099.
49. Caro FM, Alberti ML, Campins F, et al. Real-Life Experience with Pirfenidone in Idiopathic Pulmonary Fibrosis in Argentina. A Retrospective Multicenter Study. Arch Bronconeumol. 2019;55(2):75–80. doi: 10.1016/j.arbres.2018.06.014.
50. Song JW, Ogura T, Inoue Y, et al. Long-term treatment with nintedanib in Asian patients with idiopathic pulmonary fibrosis: Results from INPULSIS®-ON. Respirology. 2020;25(4):410–416. doi: 10.1111/resp.13647.
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Cilli A, Uzer F. Disease progression in idiopathic pulmonary fibrosis under anti-fibrotic treatment. Sarcoidosis Vasc Diffuse Lung Dis [Internet]. 2023 Sep. 13 [cited 2024 Nov. 24];40(3):e2023034. Available from: https://mattioli1885journals.com/index.php/sarcoidosis/article/view/14048
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How to Cite
1.
Cilli A, Uzer F. Disease progression in idiopathic pulmonary fibrosis under anti-fibrotic treatment. Sarcoidosis Vasc Diffuse Lung Dis [Internet]. 2023 Sep. 13 [cited 2024 Nov. 24];40(3):e2023034. Available from: https://mattioli1885journals.com/index.php/sarcoidosis/article/view/14048
