进展性肺纤维化诊治进展_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

林荣梅 ,  柴燕玲

昆明医科大学第二附属医院呼吸与危重症医学科二病区（云南昆明 650000）;

Corresponding author：

柴燕玲, Email: chai_yanlin@126.com

Keywords：

DOI：

10.7507/1671-6205.202208039

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Citation： 林荣梅, 柴燕玲. 进展性肺纤维化诊治进展. Chinese Journal of Respiratory and Critical Care Medicine, 2023, 22(1): 66-70. doi: 10.7507/1671-6205.202208039 Copy

1.	Wells AU, Brown KK, Flaherty KR, et al. What's in a name? That which we call IPF, by any other name would act the same. Eur Respir J, 2018, 51(5): 1800692.
2.	George PM, Spagnolo P, Kreuter M, et al. Progressive fibrosing interstitial lung disease: clinical uncertainties, consensus recommendations, and research priorities. Lancet Respir Med, 2020, 8(9): 925-934.
3.	Ryerson CJ, Kolb M. The increasing mortality of idiopathic pulmonary fibrosis: fact or fallacy. Eur Respir J, 2018, 51(1): 1702420.
4.	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, 2022, 205(9): e18-e47.
5.	Wijsenbeek M, Kreuter M, Olson A, et al. Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management. Curr Med Res Opin, 2019, 35(11): 2015-2024.
6.	Albera C, Verri G, Sciarrone F, et al. Progressive fibrosing interstitial lung diseases: a current perspective. Biomedicines, 2021, 9(9): 1237.
7.	Wijsenbeek M, Cottin V. Spectrum of fibrotic lung diseases. N Engl J Med, 2020, 383(10): 958-968.
8.	Walsh SLF, Lederer DJ, Ryerson CJ, et al. Diagnostic likelihood thresholds that define a working diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med, 2019, 200(9): 1146-1153.
9.	Wong AW, Ryerson CJ, Guler SA. Progression of fibrosing interstitial lung disease. Respir Res, 2020, 21(1): 32.
10.	Khanna D, Albera C, Fischer A, et al. An open-label, phase ii study of the safety and tolerability of pirfenidone in patients with scleroderma-associated interstitial lung disease: the LOTUSS trial. J Rheumatol, 2016, 43(9): 1672-1679.
11.	Behr J, Prasse A, Kreuter M, et al. Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial. Lancet Respir Med, 2021, 9(5): 476-486.
12.	Maher TM, Corte TJ, Fischer A, et al. Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial. Lancet Respir Med, 2020, 8(2): 147-157.
13.	Mateos-Toledo H, Mejía-Ávila M, Rodríguez-Barreto Ó, et al. An open-label study with pirfenidone on chronic hypersensitivity pneumonitis. Arch Bronconeumol (Engl Ed), 2020, 56(3): 163-169.
14.	Hilberg F, Tontsch-Grunt U, Baum A, et al. Triple angiokinase inhibitor nintedanib directly inhibits tumor cell growth and induces tumor shrinkage via blocking oncogenic receptor tyrosine kinases. J Pharmacol Exp Ther, 2018, 364(3): 494-503.
15.	Cottin V, Wollin L, Fischer A, et al. Fibrosing interstitial lung diseases: knowns and unknowns. Eur Respir Rev, 2019, 28(151): 180100.
16.	Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med, 2019, 381(18): 1718-1727.
17.	Wells AU, Flaherty KR, Brown KK, et al. Nintedanib in patients with progressive fibrosing interstitial lung diseases - subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial. Lancet Respir Med, 2020, 8(5): 453-460.
18.	Distler O, Highland KB, Gahlemann M, et al. Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med, 2019, 380(26): 2518-2528.
19.	U. S. Food and Drug Administration. OFEV®(Nintedanib Capsules), for oral use. Prescribing information[EB/OL]. (2022-02). Available at: https://docs.boehringer-ingelheim.com/Prescribing%20Information/PIs/Ofev/ofev.pdf.
20.	European Medicines Agency. Ofev. Nintedanib. EPAR—Summary of product characteristics[EB/OL]. (2020-07-28). Available at: https://www.ema.europa.eu/en/medicines/human/EPAR/ofev.
21.	Yanagihara T, Chong SG, Vierhout M, et al. Current models of pulmonary fibrosis for future drug discovery efforts. Expert Opin Drug Discov, 2020, 15(8): 931-941.
22.	Selvarajah B, Azuelos I, Anastasiou D, et al. Fibrometabolism - an emerging therapeutic frontier in pulmonary fibrosis. Sci Signal, 2021, 14(697): eaay1027.
23.	Raghu G, van den Blink B, Hamblin MJ, et al. Long-term treatment with recombinant human pentraxin 2 protein in patients with idiopathic pulmonary fibrosis: an open-label extension study. Lancet Respir Med, 2019, 7(8): 657-664.
24.	Richeldi L, Fernández Pérez ER, Costabel U, et al. Pamrevlumab, an anti-connective tissue growth factor therapy, for idiopathic pulmonary fibrosis (PRAISE): a phase 2, randomised, double-blind, placebo-controlled trial. Lancet Respir Med, 2020, 8(1): 25-33.
25.	d'alessandro M, De Vita E, Bergantini L, et al. Galactin-1, 3 and 9: potential biomarkers in idiopathic pulmonary fibrosis and other interstitial lung diseases. Respir Physiol Neurobiol, 2020, 282: 103546.
26.	Hirani N, MacKinnon AC, Nicol L, et al. Target inhibition of galectin-3 by inhaled TD139 in patients with idiopathic pulmonary fibrosis. Eur Respir J, 2021, 57(5): 2002559.
27.	Roofeh D, Lin CJF, Goldin J, et al. Tocilizumab prevents progression of early systemic sclerosis-associated interstitial lung disease. Arthritis Rheumatol, 2021, 73(7): 1301-1310.
28.	Yang SN, Liu PP, Jiang YL, et al. Therapeutic applications of mesenchymal stem cells in idiopathic pulmonary fibrosis. Front Cell Dev Biol, 2021, 9: 639657.
29.	Cottin V, Richeldi L, Rosas I, et al. Nintedanib and immunomodulatory therapies in progressive fibrosing interstitial lung diseases. Respir Res, 2021, 22(1): 84.
30.	Highland KB, Distler O, Kuwana M, et al. Efficacy and safety of nintedanib in patients with systemic sclerosis-associated interstitial lung disease treated with mycophenolate: a subgroup analysis of the SENSCIS trial. Lancet Respir Med, 2021, 9(1): 96-106.
31.	Shochet GE, Pomerantz A, Shitrit D, et al. Galectin-3 levels are elevated following nintedanib treatment. Ther Adv Chronic Dis, 2020, 11: 2040622320968412.
32.	Kreuter M, Bendstrup E, Russell AM, et al. Palliative care in interstitial lung disease: living well. Lancet Respir Med, 2017, 5(12): 968-980.
33.	Lim RK, Humphreys C, Morisset J, et al. Oxygen in patients with fibrotic interstitial lung disease: an international Delphi survey. Eur Respir J, 2019, 54(2): 1900421.
34.	Visca D, Mori L, Tsipouri V, et al. Effect of ambulatory oxygen on quality of life for patients with fibrotic lung disease (AmbOx): a prospective, open-label, mixed-method, crossover randomised controlled trial. Lancet Respir Med, 2018, 6(10): 759-770.
35.	Wijsenbeek MS, Holland AE, Swigris JJ, et al. Comprehensive supportive care for patients with fibrosing interstitial lung disease. Am J Respir Crit Care Med, 2019, 200(2): 152-159.
36.	Chambers DC, Cherikh WS, Harhay MO, et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Thirty-sixth adult lung and heart-lung transplantation Report-2019; Focus theme: Donor and recipient size match. J Heart Lung Transplant, 2019, 38(10): 1042-1055.
37.	练巧燕, 徐鑫, 罗群, 等. 肺移植治疗间质性肺疾病的疗效及预后影响因素分析. 中国呼吸与危重监护杂志, 2019, 18(2): 134-139.
38.	Kapnadak SG, Raghu G. Lung transplantation for interstitial lung disease. Eur Respir Rev, 2021, 30(161): 210017.
39.	George PM, Patterson CM, Reed AK, et al. Lung transplantation for idiopathic pulmonary fibrosis. Lancet Respir Med, 2019, 7(3): 271-282.

1. Wells AU, Brown KK, Flaherty KR, et al. What's in a name? That which we call IPF, by any other name would act the same. Eur Respir J, 2018, 51(5): 1800692.
2. George PM, Spagnolo P, Kreuter M, et al. Progressive fibrosing interstitial lung disease: clinical uncertainties, consensus recommendations, and research priorities. Lancet Respir Med, 2020, 8(9): 925-934.
3. Ryerson CJ, Kolb M. The increasing mortality of idiopathic pulmonary fibrosis: fact or fallacy. Eur Respir J, 2018, 51(1): 1702420.
4. 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, 2022, 205(9): e18-e47.
5. Wijsenbeek M, Kreuter M, Olson A, et al. Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management. Curr Med Res Opin, 2019, 35(11): 2015-2024.
6. Albera C, Verri G, Sciarrone F, et al. Progressive fibrosing interstitial lung diseases: a current perspective. Biomedicines, 2021, 9(9): 1237.
7. Wijsenbeek M, Cottin V. Spectrum of fibrotic lung diseases. N Engl J Med, 2020, 383(10): 958-968.
8. Walsh SLF, Lederer DJ, Ryerson CJ, et al. Diagnostic likelihood thresholds that define a working diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med, 2019, 200(9): 1146-1153.
9. Wong AW, Ryerson CJ, Guler SA. Progression of fibrosing interstitial lung disease. Respir Res, 2020, 21(1): 32.
10. Khanna D, Albera C, Fischer A, et al. An open-label, phase ii study of the safety and tolerability of pirfenidone in patients with scleroderma-associated interstitial lung disease: the LOTUSS trial. J Rheumatol, 2016, 43(9): 1672-1679.
11. Behr J, Prasse A, Kreuter M, et al. Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial. Lancet Respir Med, 2021, 9(5): 476-486.
12. Maher TM, Corte TJ, Fischer A, et al. Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial. Lancet Respir Med, 2020, 8(2): 147-157.
13. Mateos-Toledo H, Mejía-Ávila M, Rodríguez-Barreto Ó, et al. An open-label study with pirfenidone on chronic hypersensitivity pneumonitis. Arch Bronconeumol (Engl Ed), 2020, 56(3): 163-169.
14. Hilberg F, Tontsch-Grunt U, Baum A, et al. Triple angiokinase inhibitor nintedanib directly inhibits tumor cell growth and induces tumor shrinkage via blocking oncogenic receptor tyrosine kinases. J Pharmacol Exp Ther, 2018, 364(3): 494-503.
15. Cottin V, Wollin L, Fischer A, et al. Fibrosing interstitial lung diseases: knowns and unknowns. Eur Respir Rev, 2019, 28(151): 180100.
16. Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med, 2019, 381(18): 1718-1727.
17. Wells AU, Flaherty KR, Brown KK, et al. Nintedanib in patients with progressive fibrosing interstitial lung diseases - subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial. Lancet Respir Med, 2020, 8(5): 453-460.
18. Distler O, Highland KB, Gahlemann M, et al. Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med, 2019, 380(26): 2518-2528.
19. U. S. Food and Drug Administration. OFEV®(Nintedanib Capsules), for oral use. Prescribing information[EB/OL]. (2022-02). Available at: https://docs.boehringer-ingelheim.com/Prescribing%20Information/PIs/Ofev/ofev.pdf.
20. European Medicines Agency. Ofev. Nintedanib. EPAR—Summary of product characteristics[EB/OL]. (2020-07-28). Available at: https://www.ema.europa.eu/en/medicines/human/EPAR/ofev.
21. Yanagihara T, Chong SG, Vierhout M, et al. Current models of pulmonary fibrosis for future drug discovery efforts. Expert Opin Drug Discov, 2020, 15(8): 931-941.
22. Selvarajah B, Azuelos I, Anastasiou D, et al. Fibrometabolism - an emerging therapeutic frontier in pulmonary fibrosis. Sci Signal, 2021, 14(697): eaay1027.
23. Raghu G, van den Blink B, Hamblin MJ, et al. Long-term treatment with recombinant human pentraxin 2 protein in patients with idiopathic pulmonary fibrosis: an open-label extension study. Lancet Respir Med, 2019, 7(8): 657-664.
24. Richeldi L, Fernández Pérez ER, Costabel U, et al. Pamrevlumab, an anti-connective tissue growth factor therapy, for idiopathic pulmonary fibrosis (PRAISE): a phase 2, randomised, double-blind, placebo-controlled trial. Lancet Respir Med, 2020, 8(1): 25-33.
25. d'alessandro M, De Vita E, Bergantini L, et al. Galactin-1, 3 and 9: potential biomarkers in idiopathic pulmonary fibrosis and other interstitial lung diseases. Respir Physiol Neurobiol, 2020, 282: 103546.
26. Hirani N, MacKinnon AC, Nicol L, et al. Target inhibition of galectin-3 by inhaled TD139 in patients with idiopathic pulmonary fibrosis. Eur Respir J, 2021, 57(5): 2002559.
27. Roofeh D, Lin CJF, Goldin J, et al. Tocilizumab prevents progression of early systemic sclerosis-associated interstitial lung disease. Arthritis Rheumatol, 2021, 73(7): 1301-1310.
28. Yang SN, Liu PP, Jiang YL, et al. Therapeutic applications of mesenchymal stem cells in idiopathic pulmonary fibrosis. Front Cell Dev Biol, 2021, 9: 639657.
29. Cottin V, Richeldi L, Rosas I, et al. Nintedanib and immunomodulatory therapies in progressive fibrosing interstitial lung diseases. Respir Res, 2021, 22(1): 84.
30. Highland KB, Distler O, Kuwana M, et al. Efficacy and safety of nintedanib in patients with systemic sclerosis-associated interstitial lung disease treated with mycophenolate: a subgroup analysis of the SENSCIS trial. Lancet Respir Med, 2021, 9(1): 96-106.
31. Shochet GE, Pomerantz A, Shitrit D, et al. Galectin-3 levels are elevated following nintedanib treatment. Ther Adv Chronic Dis, 2020, 11: 2040622320968412.
32. Kreuter M, Bendstrup E, Russell AM, et al. Palliative care in interstitial lung disease: living well. Lancet Respir Med, 2017, 5(12): 968-980.
33. Lim RK, Humphreys C, Morisset J, et al. Oxygen in patients with fibrotic interstitial lung disease: an international Delphi survey. Eur Respir J, 2019, 54(2): 1900421.
34. Visca D, Mori L, Tsipouri V, et al. Effect of ambulatory oxygen on quality of life for patients with fibrotic lung disease (AmbOx): a prospective, open-label, mixed-method, crossover randomised controlled trial. Lancet Respir Med, 2018, 6(10): 759-770.
35. Wijsenbeek MS, Holland AE, Swigris JJ, et al. Comprehensive supportive care for patients with fibrosing interstitial lung disease. Am J Respir Crit Care Med, 2019, 200(2): 152-159.
36. Chambers DC, Cherikh WS, Harhay MO, et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Thirty-sixth adult lung and heart-lung transplantation Report-2019; Focus theme: Donor and recipient size match. J Heart Lung Transplant, 2019, 38(10): 1042-1055.
37. 练巧燕, 徐鑫, 罗群, 等. 肺移植治疗间质性肺疾病的疗效及预后影响因素分析. 中国呼吸与危重监护杂志, 2019, 18(2): 134-139.
38. Kapnadak SG, Raghu G. Lung transplantation for interstitial lung disease. Eur Respir Rev, 2021, 30(161): 210017.
39. George PM, Patterson CM, Reed AK, et al. Lung transplantation for idiopathic pulmonary fibrosis. Lancet Respir Med, 2019, 7(3): 271-282.

Chinese Journal of Respiratory and Critical Care Medicine

进展性肺纤维化诊治进展

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