Легочные фиброзы: новые горизонты и клинические решения. II Экспертный совет «Идиопатический легочный фиброз и прогрессирующие легочные фиброзы. Реальная практика»

Легочные фиброзы: новые горизонты и клинические решения. II Экспертный совет «Идиопатический легочный фиброз и прогрессирующие легочные фиброзы. Реальная практика». Обзор симпозиума 26 октября 2024 г., Сочи, Россия. Consilium Medicum. 2025;27(3):2–15. DOI: 10.26442/20751753.2025.3.203266

© ООО «КОНСИЛИУМ МЕДИКУМ», 2025 г.

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Pulmonary fibrosis: New horizons and clinical solutions: A review. II Expert Council “Idiopathic pulmonary fibrosis and progressive pulmonary fibroses. Real Practice”. Symposium Review October 26, 2024, Sochi, Russia. Consilium Medicum. 2025;27(3):2–15. DOI: 10.26442/20751753.2025.3.203266

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Аннотация

В обзоре представлены ключевые материалы симпозиума II Экспертного совета, посвященного идиопатическому легочному фиброзу (ИЛФ) и прогрессирующим легочным фиброзам, состоявшегося 26 октября 2024 г. в Сочи. Рассмотрены последние научные достижения в понимании патогенеза ИЛФ, включая генетические аспекты и роль фибростарения. Обсуждены обновленные клинические рекомендации Европейского респираторного общества / Американского торакального общества / Японского респираторного общества / Латиноамериканской торакальной ассоциации (2022 г.) по диагностике, акцентирующие значение трансбронхиальной криобиопсии и паттернов высокоразрешающей компьютерной томографии. Особое внимание уделено новым терапевтическим подходам: антифибротическим препаратам (нинтеданибу, пирфенидону), ингаляционным формам, а также перспективным лекарственным средствам в фазе клинических исследований. Представлены результаты исследований по оценке прогностической значимости симптомов (кашель, крепитация) и эффективности биоэквивалентных препаратов. Отражены данные Российского регистра пациентов с ИЛФ, подчеркивающие важность стандартизации диагностики и мониторинга. Материал включает клинический разбор случая, демонстрирующий долгосрочную терапию пирфенидоном. Обзор предназначен для пульмонологов, ревматологов и специалистов, занимающихся интерстициальными заболеваниями легких.

Ключевые слова: идиопатический легочный фиброз, антифибротическая терапия, клинические рекомендации, пирфенидон

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This review summarizes key materials from Symposium II of the Expert Council on idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis, held on October 26, 2024, in Sochi. It highlights recent scientific advances in understanding the pathogenesis of IPF, including genetic factors and the role of fibrosenescence. Discussed are the updated clinical guidelines from the European Respiratory Society (ERS), American Thoracic Society (ATS), Japanese Respiratory Society (JRS), and Asociación Latinoamericana del Thorax (ALAT) (2022) on diagnosis, emphasizing the value of transbronchial lung cryobiopsy and high-resolution computed tomography (HRCT) patterns. Special attention is given to novel therapeutic approaches: antifibrotic drugs (nintedanib, pirfenidone), inhaled formulations, and promising agents in clinical trial phases. Research findings on the prognostic significance of symptoms (cough, crackles) and the efficacy of bioequivalent drugs are reviewed. Data from the Russian IPF patient registry underscore the importance of standardized diagnostics and monitoring. The material includes a clinical case analysis demonstrating long-term pirfenidone therapy. This review is intended for pulmonologists, rheumatologists, and specialists in interstitial lung diseases.

Keywords: idiopathic pulmonary fibrosis, antifibrotic therapy, clinical guidelines, pirfenidone

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Список литературы

1. Chaudhuri N, Spagnolo P, Valenzuela C, et al. Treatment patterns and patient journey in progressive pulmonary fibrosis. Respir Res. 2024;25(1):364. DOI:10.1186/s12931-024-02995-9
2. Selman M, Pardo A. Idiopathic pulmonary fibrosis: From common microscopy to single-cell biology and precision medicine. Am J Respir Crit Care Med. 2024;209(9):1074-81. DOI:10.1164/rccm.202309-1573PP
3. Adegunsoye A, Kropski JA, Behr J, et al. Genetics and genomics of pulmonary fibrosis: Charting the molecular landscape and shaping precision medicine. Am J Respir Crit Care Med. 2024;210(4):401-23. DOI:10.1164/rccm.202401-0238SO
4. Chikina S, Cherniak A, Merzhoeva Z, et al. Russian Registry of idiopathic pulmonary fibrosis: Clinical features, treatment management, and outcomes. Life (Basel). 2023;13(2):435. DOI:10.3390/life13020435
5. Liao YW, Chen YM, Liu MC, et al. Multidisciplinary-derived clinical score for accurate prediction of long-term mortality in fibrotic lung disease patients. Eur J Med Res. 2024;29(1):69. DOI:10.1186/s40001-024-01644-7
6. Khor YH, Johannson KA, Marcoux V, et al. Epidemiology and prognostic significance of cough in fibrotic interstitial lung disease. Am J Respir Crit Care Med. 2024;210(8):1035-44. DOI:10.1164/rccm.202311-2101OC
7. Wu Z, Smith DJF, Yazbeck L, et al. Cough Severity Visual Analog Scale assesses cough burden and predicts survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2024;209(9):1165-7. DOI:10.1164/rccm.202311-2169LE
8. Fujita K, Kanemitsu Y, Ohkubo H, et al. Productive cough associated with patient-reported outcomes and computed tomography analysis results in idiopathic pulmonary fibrosis. ERJ Open Res. 2024;10(5):00527-2024. DOI:10.1183/23120541.00527-2024
9. Sgalla G, Simonetti J, Di Bartolomeo A, et al. Reliability of crackles in fibrotic interstitial lung disease. Respir Res. 2024;25(1):352. DOI:10.1186/s12931-024-02979-9
10. Hozumi H, Miyashita K, Nakatani E, et al. Antifibrotics and mortality in idiopathic pulmonary fibrosis: External validity and avoidance of immortal time bias. Respir Res. 2024;25(1):293. DOI:10.1186/s12931-024-02922-y
11. Romero Ortiz AD, Jiménez-Rodríguez BM, López-Ramírez C, et al. Antifibrotic treatment adherence, efficacy and outcomes for patients with idiopathic pulmonary fibrosis in Spain. BMJ Open Respir Res. 2024;11(1):e001687. DOI:10.1136/bmjresp-2023-001687
12. Richeldi L, Azuma A, Cottin V, et al. Design of a phase III, double-blind, randomised, placebo-controlled trial of BI 1015550 in patients with idiopathic pulmonary fibrosis (FIBRONEER-IPF). BMJ Open Resp Res. 2023;10(1):e001563. DOI:10.1136/bmjresp-2022-001563
13. Lancaster L, Cottin V, Ramaswamy M, et al. Bexotegrast in Patients with Idiopathic Pulmonary Fibrosis: The INTEGRIS-IPF Study. Am J Respir Crit Care Med. 2024;210(4):424-34. DOI:10.1164/rccm.202403-0636OC
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20. 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:e18-47. DOI:10.1164/rccm.202202-0399ST
21. Rajan SK, Cottin V, Dhar R, et al. Progressive pulmonary fibrosis: An expert group consensus statement. Eur Respir J. 2023;61(3):2103187. DOI:10.1183/13993003.03187-2021
22. Shigeki M. Progressive fibrosing interstitial lung diseases: A new concept and indication of Nintedanib. Mod Rheumatol. 2020;31(1):13-9. DOI:10.1080/14397595.2020.1826665
23. Kuwana M, Azuma A. Nintedanib: New indication for systemic sclerosis-associated interstitial lung disease. Mod Rheumatol. 2020;30(2):225-31. DOI:10.1080/14397595.2019.1696505
24. 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):476-86. DOI:10.1016/S2213-2600(20)30554-3
25. Solomon JJ, Danoff SK, Woodhead FA, et al. Safety, tolerability, and efficacy of pirfenidone in patients with rheumatoid arthritis-associated interstitial lung disease: A randomised, double-blind, placebo-controlled, phase 2 study. Lancet Respir Med. 2023;11(1):87-96. DOI:10.1016/S2213-2600(22)00260-0
26. 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):476-86. DOI:10.1016/S2213-2600(20)30554-3
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1. Chaudhuri N, Spagnolo P, Valenzuela C, et al. Treatment patterns and patient journey in progressive pulmonary fibrosis. Respir Res. 2024;25(1):364. DOI:10.1186/s12931-024-02995-9
2. Selman M, Pardo A. Idiopathic pulmonary fibrosis: From common microscopy to single-cell biology and precision medicine. Am J Respir Crit Care Med. 2024;209(9):1074-81. DOI:10.1164/rccm.202309-1573PP
3. Adegunsoye A, Kropski JA, Behr J, et al. Genetics and genomics of pulmonary fibrosis: Charting the molecular landscape and shaping precision medicine. Am J Respir Crit Care Med. 2024;210(4):401-23. DOI:10.1164/rccm.202401-0238SO
4. Chikina S, Cherniak A, Merzhoeva Z, et al. Russian Registry of idiopathic pulmonary fibrosis: Clinical features, treatment management, and outcomes. Life (Basel). 2023;13(2):435. DOI:10.3390/life13020435
5. Liao YW, Chen YM, Liu MC, et al. Multidisciplinary-derived clinical score for accurate prediction of long-term mortality in fibrotic lung disease patients. Eur J Med Res. 2024;29(1):69. DOI:10.1186/s40001-024-01644-7
6. Khor YH, Johannson KA, Marcoux V, et al. Epidemiology and prognostic significance of cough in fibrotic interstitial lung disease. Am J Respir Crit Care Med. 2024;210(8):1035-44. DOI:10.1164/rccm.202311-2101OC
7. Wu Z, Smith DJF, Yazbeck L, et al. Cough Severity Visual Analog Scale assesses cough burden and predicts survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2024;209(9):1165-7. DOI:10.1164/rccm.202311-2169LE
8. Fujita K, Kanemitsu Y, Ohkubo H, et al. Productive cough associated with patient-reported outcomes and computed tomography analysis results in idiopathic pulmonary fibrosis. ERJ Open Res. 2024;10(5):00527-2024. DOI:10.1183/23120541.00527-2024
9. Sgalla G, Simonetti J, Di Bartolomeo A, et al. Reliability of crackles in fibrotic interstitial lung disease. Respir Res. 2024;25(1):352. DOI:10.1186/s12931-024-02979-9
10. Hozumi H, Miyashita K, Nakatani E, et al. Antifibrotics and mortality in idiopathic pulmonary fibrosis: External validity and avoidance of immortal time bias. Respir Res. 2024;25(1):293. DOI:10.1186/s12931-024-02922-y
11. Romero Ortiz AD, Jiménez-Rodríguez BM, López-Ramírez C, et al. Antifibrotic treatment adherence, efficacy and outcomes for patients with idiopathic pulmonary fibrosis in Spain. BMJ Open Respir Res. 2024;11(1):e001687. DOI:10.1136/bmjresp-2023-001687
12. Richeldi L, Azuma A, Cottin V, et al. Design of a phase III, double-blind, randomised, placebo-controlled trial of BI 1015550 in patients with idiopathic pulmonary fibrosis (FIBRONEER-IPF). BMJ Open Resp Res. 2023;10(1):e001563. DOI:10.1136/bmjresp-2022-001563
13. Lancaster L, Cottin V, Ramaswamy M, et al. Bexotegrast in Patients with Idiopathic Pulmonary Fibrosis: The INTEGRIS-IPF Study. Am J Respir Crit Care Med. 2024;210(4):424-34. DOI:10.1164/rccm.202403-0636OC
14. ERS Congress 2024 – Annual Congress of the European Respiratory Society. Vienna, 2024.
15. Corte TJ, Behr J, Cottin V, et al. Efficacy and safety of Admilparant, an LPA1 antagonist in pulmonary fibrosis. Am J Respir Crit Care Med. 2025;211(2):230-8. DOI:10.1164/rccm.202405-0977OC
16. Nathan SD, Behr J, Cottin V, et al. Study design and rationale for the TETON-PPF Phase 3, randomized, controlled clinical trial of inhaled treprostinil in the treatment of progressive pulmonary fibrosis. CHEST Pulmonary. 2024;3(2):100124. DOI:10.1016/j.chpulm.2024.100124
17. Waxman A, Restrepo-Jaramillo R, Thenappan T, et al. Inhaled Treprostinil in pulmonary hypertension due to interstitial lung disease. N Engl J Med. 2021;384(4):325-34. DOI:10.1056/NEJMoa2008470
18. Nathan SD, Behr J, Cottin V, et al. Study design and rationale for the TETON phase 3, randomised, controlled clinical trials of inhaled treprostinil in the treatment of idiopathic pulmonary fibrosis. BMJ Open Respir Res. 2022;9(1):e001310. DOI:10.1136/bmjresp-2022-001310
19. Wu Z, Spencer LG, Banya W, et al. Morphine for treatment of cough in idiopathic pulmonary fibrosis (PACIFY COUGH). Lancet Respir Med. 2024;12(4):273-80. DOI:10.1016/S2213-2600(23)00432-0
20. 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:e18-47. DOI:10.1164/rccm.202202-0399ST
21. Rajan SK, Cottin V, Dhar R, et al. Progressive pulmonary fibrosis: An expert group consensus statement. Eur Respir J. 2023;61(3):2103187. DOI:10.1183/13993003.03187-2021
22. Shigeki M. Progressive fibrosing interstitial lung diseases: A new concept and indication of Nintedanib. Mod Rheumatol. 2020;31(1):13-9. DOI:10.1080/14397595.2020.1826665
23. Kuwana M, Azuma A. Nintedanib: New indication for systemic sclerosis-associated interstitial lung disease. Mod Rheumatol. 2020;30(2):225-31. DOI:10.1080/14397595.2019.1696505
24. 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):476-86. DOI:10.1016/S2213-2600(20)30554-3
25. Solomon JJ, Danoff SK, Woodhead FA, et al. Safety, tolerability, and efficacy of pirfenidone in patients with rheumatoid arthritis-associated interstitial lung disease: A randomised, double-blind, placebo-controlled, phase 2 study. Lancet Respir Med. 2023;11(1):87-96. DOI:10.1016/S2213-2600(22)00260-0
26. 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):476-86. DOI:10.1016/S2213-2600(20)30554-3
27. Rukovodstvo po ekspertize lekarstvennykh sredstv. Moscow: Grif i K, 2014. T. I (in Russian).

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