Идиопатический легочный фиброз: клинический случай длительного наблюдения

Игнатова Г.Л., Блинова Е.В, Бельснер М.С., Короткая М.А. Идиопатический легочный фиброз: клинический случай длительного наблюдения. Consilium Medicum. 2022;24(9):640–644.
DOI: 10.26442/20751753.2022.9.201962

© ООО «КОНСИЛИУМ МЕДИКУМ», 2022 г.

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Ignatova GL, Blinova EV, Belsner MS, Korotkaia MA. Idiopathic pulmonary fibrosis: a clinical case of long-term follow-up. Consilium Medicum. 2022;24(9):640–644. DOI: 10.26442/20751753.2022.9.201962

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Аннотация

Идиопатический легочный фиброз является особой формой хронической прогрессирующей фиброзирующей интерстициальной пневмонии неизвестной этиологии. В течение последнего десятилетия достигнут значительный прогресс в разработке и применении диагностического алгоритма для пациентов с идиопатическим легочным фиброзом, предполагающий анализ клинических, лабораторных и инструментальных данных, прежде всего результатов высокоразрешающей компьютерной томографии органов грудной клетки. Раннее назначение антифиброзной терапии позволяет оказать влияние на течение заболевания, замедлить прогрессирование патологического процесса и улучшить прогноз.

Ключевые слова: идиопатический легочный фиброз, антифибротическая терапия, пирфенидон, клинический случай

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Idiopathic pulmonary fibrosis is a specific form of chronic progressive fibrotic interstitial pneumonia of unknown etiology. Over the past decade, significant progress has been made in developing and utilizing a diagnostic algorithm for patients with idiopathic pulmonary fibrosis, involving analysis of clinical, laboratory, and instrumental data, primarily the results of high-resolution chest computed tomography. Early antifibrotic therapy can alter the disease course, slow its progression, and improve the prognosis.

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

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Список литературы

1. Российское Респираторное Общество. Идиопатический легочный фиброз. Клинические рекомендации, 2021 [Rossiiskoe Respiratornoe Obshchestvo. Idiopaticheskii legochnyi fibroz. Klinicheskie rekomendatsii, 2021 (in Russian)].
2. Raghu G, Remy-Jardin M, Myers JL, et al. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2018;198(5):e44-68. DOI:10.1164/rccm.201807-1255ST
3. Valeyre D, Duchemann B, Nunes H, et al. Interstitial Lung diseases. Respiratory epidemiology. Eur Respir Monogr. 2014;65:79-87. DOI:10.1183/2312508X.erm6514
4. Чучалин А.Г., Авдеев С.Н., Айсанов З.Р., и др. Диагностика и лечение идиопатического легочного фиброза. Федеральные клинические рекомендации. Пульмонология. 2016;26(4):399-419 [Chuchalin AG, Avdeev SN, Aisanov ZR, et al. Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis. Federal Guidelines. PULMONOLOGIYA. 2016;26(4):399-419 (in Russian)]. DOI:10.18093/0869-0189-2016-26-4-399-419
5. Чикина С.Ю., Черняк А.В., Мержоева З.М., и др. Регистр больных идиопатическим легочным фиброзом в Российской Федерации. Пульмонология. 2020;30(2):173-83 [Chikina SYu, Chernyak AV, Merzhoeva ZM, et al. Idiopathic pulmonary fibrosis Registry in Russia. PULMONOLOGIYA. 2020;30(2):173-83 (in Russian)]. DOI:10.18093/0869-0189-2020-30-2-173-183
6. Richeldi L, Rubin AS, Avdeev S, et al. Idiopathic Pulmonary Fibrosis in BRIC countries: the cases of Brazil, Russia, India, and China. BMC Med. 2015;13:237. DOI:10.1186/s12916-015-0495-0
7. Costabel U, Crestani B, Wells AU, eds. Idiopathic Pulmonary Fibrosis. ERS Monograph. 2016. DOI:10.1183/2312508X.erm7116
8. Raghu G, Collard HR, Egan JJ, et al; ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. 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
9. Sgalla G, Walsh SLF, Sverzellati N, et al. “Velcro-type” crackles predict specific radiologic features of fibrotic interstitial lung disease. BMC Pulm Med. 2018;18(1):103. DOI:10.1186/s12890-018-0670-0
10. Mathieson JR, Mayo JR, Staples CA, Müller NL. Chronic diffuse infiltrative lung disease: comparison of diagnostic accuracy of CT and chest radiography. Radiology. 1989;171(1):111-6. DOI:10.1148/radiology.171.1.2928513
11. Lynch DA, Godwin JD, Safrin S, et al. High-resolution computed tomography in idiopathic pulmonary fibrosis: diagnosis and prognosis. Am J Resir Crit Care Med. 2005;172(4):488-93. DOI:10.1164/rccm.200412-1756OC
12. Remy-Jardin M, Giraud F, Remy J, et al. Importance of ground-glass attenuation in chronic diffuse infiltrative lung disease: pathologic-CT correlation. Radiology. 1993;189(3):693-8. DOI:10.1148/radiology.189.3.8234692
13. Skandamis A, Kani C, Markantonis SL, Souliotis K. Systematic review and network meta-analysis of approved medicines for the treatment of idiopathic pulmonary fibrosis. J Drug Assess. 2019;8(1):55-61. DOI:10.1080/21556660.2019.1597726
14. King TE Jr, Bradford WZ, Castro-Bernardini S, et al; ASCEND Study Group. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083-92. DOI:10.1056/NEJMoa1402582
15. Richeldi L, du Bois RM, Raghu G, et al; INPULSIS Trial Investigators. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071-82. DOI:10.1056/ NEJMoa1402584
16. Navaratnam V, Fleming KM, West J, et al. The rising incidence of idiopathic pulmonary fibrosis in the U.K. Thorax. 2011;66(6):462-7. DOI:10.1136/thx.2010.148031
17. Raghu G, Chen SY, Yeh WS, et al. Idiopathic pulmonary fibrosis in US Medicare beneficiaries aged 65 years and older: incidence, prevalence, and survival, 2001–11. Lancet Respir Med. 2014;2(7):566-72. DOI:10.1016/S2213-2600(14)70101-8
18. Macconi D, Benigni A, Remuzzi G. The onset and Resolution of Renal Fibrosis: A Human Perspective. Kidney development, disease, repair and regeneration. Ed MH Little. Amsterdam: Elsevier Academic Press, 2016, p. 351-66. DOI:10.1016/B978-0-12-800102-8.00026-6
19. Fernandez IE, Eickelberg O. The impact of TGF-β on lung fibrosis: from targeting to biomarkers. Proc Am Thorac Soc. 2012;9(3):111-6. DOI:10.1513/pats.201203-023AW
20. Noble PW, Albera C, Bradford WZ, et al; CAPACITY Study Group. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377(9779):1760-9. DOI:10.1016/SO140-6736(11)60405-4
21. Nathan SD, Albera C, Bradford WZ, et al. Effect of pirfenidone on mortality: pooled analyses and meta-analyses of clinical trials in idiopathic pulmonary fibrosis. Lancet Respir Med. 2017;5(1):33-41. DOI:10.1016/S2213-2600(16)30326-5
22. Costabel U, Albera C, Bradford WZ, et al. Analysis of lung function and survival in RECAP: An open-label extension study of pirfenidone in patients with idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis. 2014;31(3):198-205.
23. Khanna D, Albera C, Fisher A, et al. An Open-label, Phase II Study of the Safety and Tolerability of Pirfenidone in Patients with Scleroderma-associated Interstitial Lung Dease: the LOTUSS Trial. J Rheumatol. 2016;43(9):1672-9. DOI:10.3899/jrheum.151322
24. Lancaster L, Albera C, Bradford WZ, et al. Safety of pirfenidone in patients with idiopathic pulmonary fibrosis: integrated analysis of cumulative data from 5 clinical trials. BMJ Open Respir Res. 2016;3(1):e000105. DOI:10.1136/bmjresp-2015-000105
25. Открытое, рандомизированное, перекрестное исследование сравнительной фармакокинетики и биоэквивалентности препарата Пирфенидон, капсулы, 267 мг (Аспектус Фарма, Россия) и препарата Эсбриет®, капсулы, 267 мг (Ф.Хоффманн-Ля Рош Лтд, Швейцария) с участием здоровых добровольцев. Протокол №EUS-PRF1 от 14 ноября 2019 г. [Otkrytoe, randomizirovannoe, perekrestnoe issledovanie sravnitel'noi farmakokinetiki i bioekvivalentnosti preparata Pirfenidon, kapsuly, 267 mg (Aspektus Farma, Rossiia) i preparata Esbriet®, kapsuly, 267 mg (F.Khoffmann-Lia Rosh Ltd, Shveitsariia) s uchastiem zdorovykh dobrovol'tsev. Protokol №EUS-PRF1 ot 14 noiabria 2019 g. (in Russian)].

________________________________________________

1. Rossiiskoe Respiratornoe Obshchestvo. Idiopaticheskii legochnyi fibroz. Klinicheskie rekomendatsii, 2021 (in Russian).
2. Raghu G, Remy-Jardin M, Myers JL, et al. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2018;198(5):e44-68. DOI:10.1164/rccm.201807-1255ST
3. Valeyre D, Duchemann B, Nunes H, et al. Interstitial Lung diseases. Respiratory epidemiology. Eur Respir Monogr. 2014;65:79-87. DOI:10.1183/2312508X.erm6514
4. Chuchalin AG, Avdeev SN, Aisanov ZR, et al. Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis. Federal Guidelines. PULMONOLOGIYA. 2016;26(4):399-419 (in Russian). DOI:10.18093/0869-0189-2016-26-4-399-419
5. Chikina SYu, Chernyak AV, Merzhoeva ZM, et al. Idiopathic pulmonary fibrosis Registry in Russia. PULMONOLOGIYA. 2020;30(2):173-83 (in Russian). DOI:10.18093/0869-0189-2020-30-2-173-183
6. Richeldi L, Rubin AS, Avdeev S, et al. Idiopathic Pulmonary Fibrosis in BRIC countries: the cases of Brazil, Russia, India, and China. BMC Med. 2015;13:237. DOI:10.1186/s12916-015-0495-0
7. Costabel U, Crestani B, Wells AU, eds. Idiopathic Pulmonary Fibrosis. ERS Monograph. 2016. DOI:10.1183/2312508X.erm7116
8. Raghu G, Collard HR, Egan JJ, et al; ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. 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
9. Sgalla G, Walsh SLF, Sverzellati N, et al. “Velcro-type” crackles predict specific radiologic features of fibrotic interstitial lung disease. BMC Pulm Med. 2018;18(1):103. DOI:10.1186/s12890-018-0670-0
10. Mathieson JR, Mayo JR, Staples CA, Müller NL. Chronic diffuse infiltrative lung disease: comparison of diagnostic accuracy of CT and chest radiography. Radiology. 1989;171(1):111-6. DOI:10.1148/radiology.171.1.2928513
11. Lynch DA, Godwin JD, Safrin S, et al. High-resolution computed tomography in idiopathic pulmonary fibrosis: diagnosis and prognosis. Am J Resir Crit Care Med. 2005;172(4):488-93. DOI:10.1164/rccm.200412-1756OC
12. Remy-Jardin M, Giraud F, Remy J, et al. Importance of ground-glass attenuation in chronic diffuse infiltrative lung disease: pathologic-CT correlation. Radiology. 1993;189(3):693-8. DOI:10.1148/radiology.189.3.8234692
13. Skandamis A, Kani C, Markantonis SL, Souliotis K. Systematic review and network meta-analysis of approved medicines for the treatment of idiopathic pulmonary fibrosis. J Drug Assess. 2019;8(1):55-61. DOI:10.1080/21556660.2019.1597726
14. King TE Jr, Bradford WZ, Castro-Bernardini S, et al; ASCEND Study Group. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083-92. DOI:10.1056/NEJMoa1402582
15. Richeldi L, du Bois RM, Raghu G, et al; INPULSIS Trial Investigators. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071-82. DOI:10.1056/ NEJMoa1402584
16. Navaratnam V, Fleming KM, West J, et al. The rising incidence of idiopathic pulmonary fibrosis in the U.K. Thorax. 2011;66(6):462-7. DOI:10.1136/thx.2010.148031
17. Raghu G, Chen SY, Yeh WS, et al. Idiopathic pulmonary fibrosis in US Medicare beneficiaries aged 65 years and older: incidence, prevalence, and survival, 2001–11. Lancet Respir Med. 2014;2(7):566-72. DOI:10.1016/S2213-2600(14)70101-8
18. Macconi D, Benigni A, Remuzzi G. The onset and Resolution of Renal Fibrosis: A Human Perspective. Kidney development, disease, repair and regeneration. Ed MH Little. Amsterdam: Elsevier Academic Press, 2016, p. 351-66. DOI:10.1016/B978-0-12-800102-8.00026-6
19. Fernandez IE, Eickelberg O. The impact of TGF-β on lung fibrosis: from targeting to biomarkers. Proc Am Thorac Soc. 2012;9(3):111-6. DOI:10.1513/pats.201203-023AW
20. Noble PW, Albera C, Bradford WZ, et al; CAPACITY Study Group. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377(9779):1760-9. DOI:10.1016/SO140-6736(11)60405-4
21. Nathan SD, Albera C, Bradford WZ, et al. Effect of pirfenidone on mortality: pooled analyses and meta-analyses of clinical trials in idiopathic pulmonary fibrosis. Lancet Respir Med. 2017;5(1):33-41. DOI:10.1016/S2213-2600(16)30326-5
22. Costabel U, Albera C, Bradford WZ, et al. Analysis of lung function and survival in RECAP: An open-label extension study of pirfenidone in patients with idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis. 2014;31(3):198-205.
23. Khanna D, Albera C, Fisher A, et al. An Open-label, Phase II Study of the Safety and Tolerability of Pirfenidone in Patients with Scleroderma-associated Interstitial Lung Dease: the LOTUSS Trial. J Rheumatol. 2016;43(9):1672-9. DOI:10.3899/jrheum.151322
24. Lancaster L, Albera C, Bradford WZ, et al. Safety of pirfenidone in patients with idiopathic pulmonary fibrosis: integrated analysis of cumulative data from 5 clinical trials. BMJ Open Respir Res. 2016;3(1):e000105. DOI:10.1136/bmjresp-2015-000105
25. Otkrytoe, randomizirovannoe, perekrestnoe issledovanie sravnitel'noi farmakokinetiki i bioekvivalentnosti preparata Pirfenidon, kapsuly, 267 mg (Aspektus Farma, Rossiia) i preparata Esbriet®, kapsuly, 267 mg (F.Khoffmann-Lia Rosh Ltd, Shveitsariia) s uchastiem zdorovykh dobrovol'tsev. Protokol №EUS-PRF1 ot 14 noiabria 2019 g. (in Russian).

Авторы

Г.Л. Игнатова1, Е.В. Блинова*1, М.С. Бельснер1, М.А. Короткая2

1 ФГБОУ ВО «Южно-Уральский государственный медицинский университет» Минздрава России, Челябинск, Россия;
2 ГАУЗ «Областная клиническая больница №3», Челябинск, Россия
*blinel@mail.ru

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Galina L. Ignatova1, Elena V. Blinova*1, Maria S. Belsner1, Marina A. Korotkaia2

1 South Ural State Medical University, Chelyabinsk, Russia;
2 Regional Clinical Hospital №3, Chelyabinsk, Russia
*blinel@mail.ru

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