medsovetМедицинский СоветMeditsinskiy sovet = Medical Council2079-701X2658-5790REMEDIUM GROUP Ltd.10.21518/ms2024-105medsovet-8325Research ArticleХРОНИЧЕСКИЕ ЗАБОЛЕВАНИЯ ЛЕГКИХCHRONIC PULMONARY DISEASESЧто изменила антифибротическая терапия для пациентов с идиопатическим легочным фиброзомWhat has antifibrotic therapy changed for patients with idiopathic pulmonary fibrosishttps://orcid.org/0000-0002-1166-9717КузубоваН. А.KuzubovaN. A.

Кузубова Наталия Анатольевна - д.м.н., начальник Экспертного центра по профилю «Пульмонология».

194354, Санкт-Петербург, Учебный переулок, д. 5

Natalia A. Kuzubova - Dr. Sci. (Med.), Head of the Expert Center on the Profile of “Pulmonology", City Multidisciplinary Hospital No. 2.

5, Uchebnyy Lane, St Petersburg, 194354

kuzubova@mail.ruhttps://orcid.org/0000-0003-4678-3904ТитоваО. Н.TitovaO. N.

Титова Ольга Николаевна - д.м.н., профессор, директор, Научно-исследовательский институт пульмонологии.

197022, Санкт-Петербург, ул. Льва Толстого, д. 6–8

Olga N. Titova - Dr. Sci. (Med.), Professor, Director of Research Institute of Pulmonology, Pavlov First Saint Petersburg State Medical University.

6–8, Lev Tolstoy St., St Petersburg, 197022

titovaon@spb-gmu.ruhttps://orcid.org/0000-0001-7052-6903СкляроваД. Б.SkliarovaD. B.

Склярова Дарья Борисовна - к.м.н., заведующий амбулаторно-консультативным отделением №2 Экспертного центра по профилю «Пульмонология».

194354, Санкт-Петербург, Учебный переулок, д. 5

Daria B. Skliarova - Cand. Sci. (Med.), Head of Outpatient Department No. 2, Expert Center on the profile «Pulmonology», City Multidisciplinary Hospital No. 2.

5, Uchebnyy Lane, St Petersburg, 194354

darya_sklyarova@mail.ruhttps://orcid.org/0000-0002-5664-7386ВолчковВ. A.VolchkovV. A.

Волчков Владимир Анатольевич - д.м.н., заведующий кафедрой анестезиологии и реаниматологии медицинского факультета.

199034, Санкт-Петербург, Университетская наб., д. 7/9

Vladimir A. Volchkov - Dr. Sci. (Med.), Head of the Department of Anesthesiology and Reanimatology, St Petersburg State University.

7–9, Universitetskaya Emb., St Petersburg, 199034

Городская многопрофильная больница №2РоссияCity Multidisciplinary Hospital No. 2Russian FederationПервый Санкт-Петербургский государственный медицинский университет имени академика И.П. ПавловаРоссияPavlov First Saint Petersburg State Medical UniversityRussian FederationСанкт-Петербургский государственный университетРоссияSt Petersburg State UniversityRussian Federation202405062024091925Copyright © Кузубова Н.А., Титова О.Н., Склярова Д.Б., Волчков В.A., 20242024Кузубова Н.А., Титова О.Н., Склярова Д.Б., Волчков В.A.Kuzubova N.A., Titova O.N., Skliarova D.B., Volchkov V.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.med-sovet.pro/jour/article/view/8325

Идиопатический легочный фиброз (ИЛФ) – хроническое прогрессирующее фиброзирующее интерстициальное заболевание легких неизвестной причины. ИЛФ характеризуется избыточной продукцией и отложением компонентов внеклеточного матрикса, которые приводят к необратимым нарушениям архитектоники легочной ткани и потере функции. Без лечения средняя выживаемость пациентов после постановки диагноза не превышает 3–5 лет. Однако опубликованные наблюдения сообщают об улучшении выживаемости за последнее десятилетие, что связано с появлением антифибротических препаратов и более ранней диагностикой. Преимущества антифибротической терапии включают более медленные темпы снижения форсированной жизненной емкости легких (ФЖЕЛ) и снижение смертности. Пирфенидон и нинтеданиб являются единственными одобренными в настоящее время антифибротическими препаратами для лечения ИЛФ. На российском рынке зарегистрировано несколько воспроизведенных препаратов с МНН пирфенидон, в том числе препарат ПИРФАСПЕК® (капсулы 267 мг). Их эффективность и безопасность были продемонстрированы как в рандомизированных клинических исследованиях, так и в исследованиях реальной клинической практики. Регистры пациентов с ИЛФ, ведущиеся во многих странах с 2010 г., предоставляют дополнительную информацию в отношении прогрессирования заболевания, эффективности терапии, частоты развития нежелательных явлений. Хотя они имеют разный механизм действия и профиль безопасности, их эффективность в замедлении снижения ФЖЕЛ и снижении риска смертности с течением времени аналогична. Тем не менее ИЛФ по-прежнему характеризуется прогрессирующей одышкой и плохим прогнозом, поскольку лечение может только задержать прогрессирование ИЛФ и не может остановить или обратить вспять повреждение. Хотя в настоящее время продолжаются клинические испытания новых лекарственных средств для лечения ИЛФ, никакие другие препараты в Российской Федерации пока не были одобрены.

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive fibrotic interstitial lung disease of unknown cause. IPF is characterized by excessive production and deposition of extracellular matrix components, which lead to irreversible violations of the architectonics of lung tissue and loss of function. Without treatment, the average survival rate of patients after diagnosis does not exceed 3–5 years. However, published observations report improved survival over the past decade, due to the advent of antifibrotic drugs and earlier diagnosis. The benefits of antifibrotic therapy include a slower rate of reduction in forced lung capacity (FVC) and a reduction in mortality. Pirfenidone and nantedanib are the only currently approved antifibrotic drugs for the treatment of IPF. Several generic drugs with the INN pirfenidone are registered on the Russian market, including the drug PIRFASPEC® (267 mg capsules). Their efficacy and safety have been demonstrated both in randomized clinical trials and in real clinical practice studies. IPF patient registries, which have been maintained in many countries since 2010, provide additional information regarding the progression of the disease, the effectiveness of therapy, and the frequency of adverse events. Although they have a different mechanism of action and safety profile, their effectiveness in slowing the decline of FVC and reducing the risk of mortality over time is similar. However, IPF is still characterized by progressive shortness of breath and poor prognosis, as treatment can only delay the progression of IPF and cannot stop or reverse the damage. Although clinical trials of new drugs for the treatment of IPF are currently underway, no other drugs have yet been approved in the Russian Federation.

идиопатический легочный фиброзпрогрессирующий фиброз легкихантифибротическая терапияпирфенидонбезопасностьidiopathic pulmonary fibrosisprogressive pulmonary fibrosisantifibrotic therapypirfenidonesafetyReferencesRaghu G, Remy-Jardin M, Richeldi L, Thomson CC, Inoue Y, Johkoh T 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. https://doi.org/10.1164/rccm.202202-0399ST.Raghu G, Remy-Jardin M, Richeldi L, Thomson CC, Inoue Y, Johkoh T 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. https://doi.org/10.1164/rccm.202202-0399ST.Lederer DJ, Martinez FJ. Idiopathic Pulmonary Fibrosis. N Engl J Med. 2018;378:1811–1823. https://doi.org/10.1056/NEJMra1705751.Lederer DJ, Martinez FJ. Idiopathic Pulmonary Fibrosis. N Engl J Med. 2018;378:1811–1823. https://doi.org/10.1056/NEJMra1705751.Natsuizaka M, Chiba H, Kuronuma K, Otsuka M, Kudo K, Mori M et al. Epidemiologic survey of Japanese patients with idiopathic pulmonary fibrosis and investigation of ethnic differences. Am J Respir Crit Care Med. 2014;190:773–779. https://doi.org/10.1164/rccm.201403-0566OC.Natsuizaka M, Chiba H, Kuronuma K, Otsuka M, Kudo K, Mori M et al. Epidemiologic survey of Japanese patients with idiopathic pulmonary fibrosis and investigation of ethnic differences. Am J Respir Crit Care Med. 2014;190:773–779. https://doi.org/10.1164/rccm.201403-0566OC.Schäfer SC, Funke-Chambour M, Berezowska S. Idiopathic pulmonary fibrosis-epidemiology, causes, and clinical course. Pathologe. 2020;41(1):46–51. https://doi.org/10.1007/s00292-019-00747-x.Schäfer SC, Funke-Chambour M, Berezowska S. Idiopathic pulmonary fibrosis-epidemiology, causes, and clinical course. Pathologe. 2020;41(1):46–51. https://doi.org/10.1007/s00292-019-00747-x.Чикина СЮ, Черняк АВ, Мержоева ЗМ, Тюрин ИЕ, Авдеев СН. Регистр больных идиопатическим легочным фиброзом в Российской Федерации. Пульмонология. 2020;30(2):173–183. Режим доступа: https://journal.pulmonology.ru/pulm/article/view/1295/1007.Chikina SYu, Chernyak AV, Merzhoeva ZM, Tyurin IE, Avdeev SN. Idiopathic pulmonary fibrosis Registry in Russia. Pulmonologiya. 2020;30(2):173–183. (In Russ.) Available at: https://journal.pulmonology.ru/pulm/article/view/1295.Bonella F, Spagnolo P, Ryerson C. Current and Future Treatment Landscape for Idiopathic Pulmonary Fibrosis. Drugs. 2023;83(17):1581–1593. Available at: https://doi.org/10.1007/s40265-023-01950-0.Bonella F, Spagnolo P, Ryerson C. Current and Future Treatment Landscape for Idiopathic Pulmonary Fibrosis. Drugs. 2023;83(17):1581–1593. Available at: https://doi.org/10.1007/s40265-023-01950-0.Авдеев СН, Трушенко НВ. Антифибротическая терапия идиопатического легочного фиброза: соотношение эффективности и безопасности. Медицинский совет. 2018;(15):131–136. https://doi.org/10.21518/2079-701X-2018-15-131-136.Avdeev SN, Trushenko NV. Antifibrotic therapy of idiopathic pulmonary fibrosis: efficiency / safety ratio. Meditsinskiy Sovet. 2018;(15):131–136. (In Russ.) https://doi.org/10.21518/2079-701X-2018-15-131-136.Bang AA, Bang S, Bang A, Acharya S, Shukla S. Recent Advances in the Treatment of Interstitial Lung Diseases. Cureus. 2023;15(10):e48016. https://doi.org/10.7759/cureus.48016.Bang AA, Bang S, Bang A, Acharya S, Shukla S. Recent Advances in the Treatment of Interstitial Lung Diseases. Cureus. 2023;15(10):e48016. https://doi.org/10.7759/cureus.48016.Guo H, Sun J, Zhang S, Nie Y, Zhou S, Zeng Y. Progress in understanding and treating idiopathic pulmonary fibrosis: recent insights and emerging therapies. Front Pharmacol. 2023;14:1205948. https://doi.org/10.3389/fphar.2023.1205948.Guo H, Sun J, Zhang S, Nie Y, Zhou S, Zeng Y. Progress in understanding and treating idiopathic pulmonary fibrosis: recent insights and emerging therapies. Front Pharmacol. 2023;14:1205948. https://doi.org/10.3389/fphar.2023.1205948.Nakamura Y, Shimizu Y, Fujimaki-Shiraishi M, Uchida N, Takemasa A, Niho S. A Protective Effect of Pirfenidone in Lung Fibroblast-Endothelial Cell Network via Inhibition of Rho-Kinase Activity. Biomedicines. 2023;11(8):2259. https://doi.org/10.3390/biomedicines11082259.Nakamura Y, Shimizu Y, Fujimaki-Shiraishi M, Uchida N, Takemasa A, Niho S. A Protective Effect of Pirfenidone in Lung Fibroblast-Endothelial Cell Network via Inhibition of Rho-Kinase Activity. Biomedicines. 2023;11(8):2259. https://doi.org/10.3390/biomedicines11082259.Gan D, Cheng W, Ke L, Sun AR, Jia Q, Chen J et al. Biphasic Effect of Pirfenidone on Angiogenesis. Front Pharmacol. 2021;12:804327. https://doi.org/10.3389/fphar.2021.804327.Gan D, Cheng W, Ke L, Sun AR, Jia Q, Chen J et al. Biphasic Effect of Pirfenidone on Angiogenesis. Front Pharmacol. 2021;12:804327. https://doi.org/10.3389/fphar.2021.804327.Kang JH, Yang MS, Kim DW, Park CW. In vivo pharmacokinetic and pharmacodynamic study of co-spray-dried inhalable pirfenidone microparticles in rats. Drug Deliv. 2022;29(1):3384–3396. https://doi.org/10.1080/10717544.2022.2149899.Kang JH, Yang MS, Kim DW, Park CW. In vivo pharmacokinetic and pharmacodynamic study of co-spray-dried inhalable pirfenidone microparticles in rats. Drug Deliv. 2022;29(1):3384–3396. https://doi.org/10.1080/10717544.2022.2149899.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. 2014;58:13–19. https://doi.org/10.1016/j.ejps.2014.02.014.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. 2014;58:13–19. https://doi.org/10.1016/j.ejps.2014.02.014.Song X, Yu W, Guo F. Pirfenidone suppresses bleomycin-induced pulmonary fibrosis and periostin expression in rats. Exp Ther Med. 2018;16(3):1800–1806. https://doi.org/10.3892/etm.2018.6378.Song X, Yu W, Guo F. Pirfenidone suppresses bleomycin-induced pulmonary fibrosis and periostin expression in rats. Exp Ther Med. 2018;16(3):1800–1806. https://doi.org/10.3892/etm.2018.6378.King TE Jr, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083–2092. https://doi.org/10.1056/NEJMoa1402582.King TE Jr, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083–2092. https://doi.org/10.1056/NEJMoa1402582.Noble PW, Albera C, Bradford WZ, Costabel U, Glassberg MK, Kardatzke D et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377(9779):1760–1769. https://doi.org/10.1016/S0140-6736(11)60405-4.Noble PW, Albera C, Bradford WZ, Costabel U, Glassberg MK, Kardatzke D et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377(9779):1760–1769. https://doi.org/10.1016/S0140-6736(11)60405-4.Behr J, Nathan SD, Costabel U, Albera C, Wuyts WA, Glassberg MK et al. Efficacy and Safety of Pirfenidone in Advanced Versus Non-Advanced Idiopathic Pulmonary Fibrosis: Post-Hoc Analysis of Six Clinical Studies. Adv Ther. 2023;40(9):3937–3955. https://doi.org/10.1007/s12325-023-02565-3.Behr J, Nathan SD, Costabel U, Albera C, Wuyts WA, Glassberg MK et al. Efficacy and Safety of Pirfenidone in Advanced Versus Non-Advanced Idiopathic Pulmonary Fibrosis: Post-Hoc Analysis of Six Clinical Studies. Adv Ther. 2023;40(9):3937–3955. https://doi.org/10.1007/s12325-023-02565-3.Costabel U, Albera C, Lancaster LH, Lin CY, Hormel P, Hulter HN, Noble PW. An Open-Label Study of the Long-Term Safety of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis (RECAP). Respiration. 2017;94(5):408–415. https://doi.org/10.1159/000479976.Costabel U, Albera C, Lancaster LH, Lin CY, Hormel P, Hulter HN, Noble PW. An Open-Label Study of the Long-Term Safety of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis (RECAP). Respiration. 2017;94(5):408–415. https://doi.org/10.1159/000479976.Cottin V, Koschel D, Günther A, Albera C, Azuma A, Sköld CM et al. Long-term safety of pirfenidone: results of the prospective, observational PASSPORT study. ERJ Open Research. 2018;4(4):00084–2018. https://doi.org/10.1183/23120541.00084-2018.Cottin V, Koschel D, Günther A, Albera C, Azuma A, Sköld CM et al. Long-term safety of pirfenidone: results of the prospective, observational PASSPORT study. ERJ Open Research. 2018;4(4):00084–2018. https://doi.org/10.1183/23120541.00084-2018.Mandovra NP, Vaidya PJ, Shah RS, Nighojkar AS, Chavhan VB, Lohiya A et al. Factors Affecting Best-Tolerated Dose of Pirfenidone in Patients with Fibrosing Interstitial Lung Disease. J Clin Med. 2023;12(20):6513. https://doi.org/10.3390/jcm12206513.Mandovra NP, Vaidya PJ, Shah RS, Nighojkar AS, Chavhan VB, Lohiya A et al. Factors Affecting Best-Tolerated Dose of Pirfenidone in Patients with Fibrosing Interstitial Lung Disease. J Clin Med. 2023;12(20):6513. https://doi.org/10.3390/jcm12206513.Molina-Molina M, Shull JG, Vicens-Zygmunt V, Rivera-Ortega P, Antoniou K, Bonella F et al. Gastrointestinal pirfenidone adverse events in idiopathic pulmonary fibrosis depending on diet: the MADIET clinical trial. Eur Respir J. 2023;62(4):2300262. https://doi.org/10.1183/13993003.00262-2023.Molina-Molina M, Shull JG, Vicens-Zygmunt V, Rivera-Ortega P, Antoniou K, Bonella F et al. Gastrointestinal pirfenidone adverse events in idiopathic pulmonary fibrosis depending on diet: the MADIET clinical trial. Eur Respir J. 2023;62(4):2300262. https://doi.org/10.1183/13993003.00262-2023.Moor CC, Mostard RLM, Grutters JC, Bresser P, Aerts JGJV, Dirksen CD et al. Patient expectations, experiences and satisfaction with nintedanib and pirfenidone in idiopathic pulmonary fibrosis: a quantitative study. Respir Res. 2020;21(1):196. https://doi.org/10.1186/s12931-020-01458-1.Moor CC, Mostard RLM, Grutters JC, Bresser P, Aerts JGJV, Dirksen CD et al. Patient expectations, experiences and satisfaction with nintedanib and pirfenidone in idiopathic pulmonary fibrosis: a quantitative study. Respir Res. 2020;21(1):196. https://doi.org/10.1186/s12931-020-01458-1.Behr J, Kreuter M, Hoeper MM, Wirtz H, Klotsche J, Koschel D et al. Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry. Eur Respir J. 2015;46:186–196. https://doi.org/10.1183/09031936.00217614.Behr J, Kreuter M, Hoeper MM, Wirtz H, Klotsche J, Koschel D et al. Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry. Eur Respir J. 2015;46:186–196. https://doi.org/10.1183/09031936.00217614.Ferrara G, Carlson L, Palm A, Einarsson J, Olivesten C, Sköld M. Idiopathic pulmonary fibrosis in Sweden: report from the first year of activity of the Swedish IPF-Registry. Eur Clin Respir J. 2016;3:31090. https://doi.org/10.3402/ecrj.v3.31090.Ferrara G, Carlson L, Palm A, Einarsson J, Olivesten C, Sköld M. Idiopathic pulmonary fibrosis in Sweden: report from the first year of activity of the Swedish IPF-Registry. Eur Clin Respir J. 2016;3:31090. https://doi.org/10.3402/ecrj.v3.31090.Guenther A, Krauss E, Tello S, Wagner J, Paul B, Kuhn S et al. The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis. Respir Res. 2018;19:141. https://doi.org/10.1186/s12931-018-0845-5.Guenther A, Krauss E, Tello S, Wagner J, Paul B, Kuhn S et al. The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis. Respir Res. 2018;19:141. https://doi.org/10.1186/s12931-018-0845-5.Jo HE, Glaspole I, Grainge C, Goh N, Hopkins PM, Moodley Y et al. Baseline characteristics of idiopathic pulmonary fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry. Eur Respir J. 2017;49:1601592. https://doi.org/10.1183/13993003.01592-2016.Jo HE, Glaspole I, Grainge C, Goh N, Hopkins PM, Moodley Y et al. Baseline characteristics of idiopathic pulmonary fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry. Eur Respir J. 2017;49:1601592. https://doi.org/10.1183/13993003.01592-2016.O’Brien EC, Durheim MT, Gamerman V, Garfinkel S, Anstrom KJ, Palmer SM, Conoscenti CS. Rationale for and design of the Idiopathic Pulmonary Fibrosis - PRospective Outcomes (IPF-PRO) registry. BMJ Open Respir Res. 2016;3:e000108. https://doi.org/10.1136/bmjresp-2015-000108.O’Brien EC, Durheim MT, Gamerman V, Garfinkel S, Anstrom KJ, Palmer SM, Conoscenti CS. Rationale for and design of the Idiopathic Pulmonary Fibrosis - PRospective Outcomes (IPF-PRO) registry. BMJ Open Respir Res. 2016;3:e000108. https://doi.org/10.1136/bmjresp-2015-000108.Wuyts WA, Dahlqvist C, Slabbynck H, Schlesser M, Gusbin N, Compere C et al. Longitudinal clinical outcomes in a real-world population of patients with idiopathic pulmonary fibrosis: the PROOF registry. Respir Res. 2019;20(1):231. https://doi.org/10.1186/s12931-019-1182-z.Wuyts WA, Dahlqvist C, Slabbynck H, Schlesser M, Gusbin N, Compere C et al. Longitudinal clinical outcomes in a real-world population of patients with idiopathic pulmonary fibrosis: the PROOF registry. Respir Res. 2019;20(1):231. https://doi.org/10.1186/s12931-019-1182-z.Терпигорев СА, Белевский АС. Эффективность и безопасность пирфенидона у больных идиопатическим легочным фиброзом: данные многоцентровых и локальных исследований. Практическая пульмонология. 2019;(3):56–60. Режим доступа: https://atmosphere-ph.ru/modules.php?name=Magazines&sop=viewarticle&magid=1&issueid=475&artid=5882.Terpigorev SA, Belevsky AS. Efficacy and safety of pirfenidone in patients with idiopathic pulmonary fibrosis: data from multicenter and local studies. Prakticheskaya Pulʹmonologiya. 2019;(3):56–60. (In Russ.) Available at: https://atmosphere-ph.ru/modules.php?name=Magazines&sop=viewarticle&magid=1&issueid=475&artid=5882.Jastrzębski D, Kostorz-Nosal S, Galle D, Gałeczka-Turkiewicz A, Warzecha J, Majewski S et al. Expectations, symptoms, and quality of life before and after 1 year of Pirfenidone treatment in patients with idiopathic pulmonary fibrosis: A single-arm, open-label nonrandomized study. Health Sci Rep. 2023;6(8):e1449. https://doi.org/10.1002/hsr2.1449.Jastrzębski D, Kostorz-Nosal S, Galle D, Gałeczka-Turkiewicz A, Warzecha J, Majewski S et al. Expectations, symptoms, and quality of life before and after 1 year of Pirfenidone treatment in patients with idiopathic pulmonary fibrosis: A single-arm, open-label nonrandomized study. Health Sci Rep. 2023;6(8):e1449. https://doi.org/10.1002/hsr2.1449.Ma M, Cao M, Gao Y, Qiu X, Jiang H, Cai H. Diagnostic finding on high-resolution computed tomography (HRCT) predicts a good response to pirfenidone in patients with idiopathic pulmonary fibrosis. Medicine (Baltimore). 2023;102(19):e33722. https://doi.org/10.1097/MD.0000000000033722.Ma M, Cao M, Gao Y, Qiu X, Jiang H, Cai H. Diagnostic finding on high-resolution computed tomography (HRCT) predicts a good response to pirfenidone in patients with idiopathic pulmonary fibrosis. Medicine (Baltimore). 2023;102(19):e33722. https://doi.org/10.1097/MD.0000000000033722.Bocchino M, Bruzzese D, Scioscia G, Capitelli L, Tondo P, Rea G et al. Disease stage-related survival in idiopathic pulmonary fibrosis patients treated with nintedanib and pirfenidone: An exploratory study. Respir Med Res. 2023;84:101013. https://doi.org/10.1016/j.resmer.2023.101013.Bocchino M, Bruzzese D, Scioscia G, Capitelli L, Tondo P, Rea G et al. Disease stage-related survival in idiopathic pulmonary fibrosis patients treated with nintedanib and pirfenidone: An exploratory study. Respir Med Res. 2023;84:101013. https://doi.org/10.1016/j.resmer.2023.101013.Alonso-Gonzalez A, Tosco-Herrera E, Molina-Molina M, Flores C. Idiopathic pulmonary fibrosis and the role of genetics in the era of precision medicine. Front Med (Lausanne). 2023;10:1152211. https://doi.org/10.3389/fmed.2023.1152211.Alonso-Gonzalez A, Tosco-Herrera E, Molina-Molina M, Flores C. Idiopathic pulmonary fibrosis and the role of genetics in the era of precision medicine. Front Med (Lausanne). 2023;10:1152211. https://doi.org/10.3389/fmed.2023.1152211.Kubbara A, Amundson WH, Herman A, Lee AM, Bishop JR, Kim HJ. Genetic variations in idiopathic pulmonary fibrosis and patient response to pirfenidone. Heliyon. 2023;9(8):e18573. https://doi.org/10.1016/j.heliyon.2023.e18573.Kubbara A, Amundson WH, Herman A, Lee AM, Bishop JR, Kim HJ. Genetic variations in idiopathic pulmonary fibrosis and patient response to pirfenidone. Heliyon. 2023;9(8):e18573. https://doi.org/10.1016/j.heliyon.2023.e18573.Flaherty KR, Fell CD, Huggins JT, Nunes H, Sussman R, Valenzuela C et al. Safety of nintedanib added to pirfenidone treatment for idiopathic pulmonary fibrosis. Eur Respir J. 2018;52(4):1800230. https://doi.org/10.1183/13993003.00230-2018.Flaherty KR, Fell CD, Huggins JT, Nunes H, Sussman R, Valenzuela C et al. Safety of nintedanib added to pirfenidone treatment for idiopathic pulmonary fibrosis. Eur Respir J. 2018;52(4):1800230. https://doi.org/10.1183/13993003.00230-2018.Vancheri C, Kreuter M, Richeldi L, Ryerson CJ, Valeyre D, Grutters JC et al. Nintedanib with add-on pirfenidone in idiopathic pulmonary fibrosis. Results of the INJOURNEY trial. Am J Respir Crit Care Med. 2018;197(3):356–363. https://doi.org/10.1164/rccm.201706-1301OC.Vancheri C, Kreuter M, Richeldi L, Ryerson CJ, Valeyre D, Grutters JC et al. Nintedanib with add-on pirfenidone in idiopathic pulmonary fibrosis. Results of the INJOURNEY trial. Am J Respir Crit Care Med. 2018;197(3):356–363. https://doi.org/10.1164/rccm.201706-1301OC.Huh JY, Lee JH, Song JW. Efficacy and safety of combination therapy with pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis. Front Pharmacol. 2023;14:1301923. https://doi.org/10.3389/fphar.2023.1301923.Huh JY, Lee JH, Song JW. Efficacy and safety of combination therapy with pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis. Front Pharmacol. 2023;14:1301923. https://doi.org/10.3389/fphar.2023.1301923.Ryerson CJ, Urbania TH, Richeldi L, Mooney JJ, Lee JS, Jones KD et al. Prevalence and prognosis of unclassifiable interstitial lung disease. Eur Respir J. 2013;42:750–757. https://doi.org/10.1183/09031936.00131912.Ryerson CJ, Urbania TH, Richeldi L, Mooney JJ, Lee JS, Jones KD et al. Prevalence and prognosis of unclassifiable interstitial lung disease. Eur Respir J. 2013;42:750–757. https://doi.org/10.1183/09031936.00131912.Behr J, Prasse A, Kreuter M, Johow J, Rabe KF, Bonella F 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. https://doi.org/10.1016/S2213-2600(20)30554-3.Behr J, Prasse A, Kreuter M, Johow J, Rabe KF, Bonella F 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. https://doi.org/10.1016/S2213-2600(20)30554-3.

The authors declare that there are no conflicts of interest present.