medsovetМедицинский СоветMeditsinskiy sovet = Medical Council2079-701X2658-5790REMEDIUM GROUP Ltd.10.21518/ms2025-201medsovet-9200Research ArticleИНТЕРСТИЦИАЛЬНЫЕ ЗАБОЛЕВАНИЯ ЛЕГКИХINTERSTITIAL PULMONARY DISEASESПерспективы использования пирфенидона при идиопатическом легочном фиброзе и прогрессирующих легочных фиброзахProspects of pirfenidone use in idiopathic pulmonary fibrosis and progressive pulmonary fibrosishttps://orcid.org/0000-0002-0685-4133ТрушенкоН. В.TrushenkoN. V.

Трушенко Наталья Владимировна, к.м.н., доцент кафедры пульмонологии Института клинической медицины имени Н.В. Склифосовского, Первый Московский государственный медицинский университет имени И.М. Сеченова (Сеченовский Университет); научный сотрудник, Научно-исследовательский институт пульмонологии

119991, Москва, ул. Трубецкая, д. 8, стр. 2,

115682, Москва, Ореховый бульвар, д. 28

Natalia V. Trushenko, Cand. Sci. (Med.), Associate Professor, Department of Pulmonology of Sklifosovsky Institute of Clinical Medicine, Sechenov First Moscow State Medical University (Sechenov University); Researcher, Research Institute for Pulmonology of the Federal Medical Biological Agency

8, Bldg. 2, Trubetskaya St., Moscow, 119991,

8, Orekhovy Boulevard, Moscow, 115682

trushenko.natalia@yandex.ruhttps://orcid.org/0000-0002-0928-2900ЛевинаЮ. А.LevinaYu. A.

Левина Юлия Алексеевна, ординатор кафедры пульмонологии Института клинической медицины имени Н.В. Склифосовского

119991, Москва, ул. Трубецкая, д. 8, стр. 2

Yulia A. Levina, Resident of the Department of Pulmonology of Sklifosovsky Institute of Clinical Medicine

8, Bldg. 2, Trubetskaya St., Moscow, 11999

yu1999levina@gmail.comhttps://orcid.org/0000-0003-1254-6863ЛавгиноваБ. Б.LavginovaB. B.

Лавгинова Баина Баатровна, ординатор кафедры пульмонологии Института клинической медицины имени Н.В. Склифосовского

119991, Москва, ул. Трубецкая, д. 8, стр. 2

Baina B. Lavginova, Resident of the Department of Pulmonology of Sklifosovsky Institute of Clinical Medicine

8, Bldg. 2, Trubetskaya St., Moscow, 11999

bapus15@yandex.ruhttps://orcid.org/0000-0001-9661-7213СувороваО. А.SuvorovaO. А.

Суворова Ольга Александровна, ассистент кафедры пульмонологии Института клинической медицины имени Н.В. Склифосовского

119991, Москва, ул. Трубецкая, д. 8, стр. 2

Olga A. Suvorova, Assistant, Department of Pulmonology of Sklifosovsky Institute of Clinical Medicine

8, Bldg. 2, Trubetskaya St., Moscow, 11999

olga.a.suvorova@mail.ruhttps://orcid.org/0000-0002-5999-2150АвдеевС. Н.AvdeevS. N.

Авдеев Сергей Николаевич, академик РАН, д.м.н., профессор, заведующий кафедрой пульмонологии Института клинической медицины имени Н.В. Склифосовского, Первый Московский государственный медицинский университет имени И.М. Сеченова (Сеченовский Университет); ведущий научный сотрудник Научно-исследовательского института пульмонологии; директор Национального медицинского исследовательского центра по профилю «Пульмонология», главный внештатный специалист-пульмонолог Министерства здравоохранения Российской Федерации

119991, Москва, ул. Трубецкая, д. 8, стр. 2,

115682, Москва, Ореховый бульвар, д. 28

Sergey N. Avdeev, Acad. RAS, Dr. Sci. (Med.), Professor, Head of the Department of Pulmonology of Sklifosovsky Institute of Clinical Medicine, Sechenov First Moscow State Medical University (Sechenov University); Leading Researcher of the Research Institute for Pulmonology of the Federal Medical Biological Agency; Director of the National Medical Research Center for Pulmonology, Chief Outside Specialist Pulmonologist of the Ministry of Health of the Russian Federation

8, Bldg. 2, Trubetskaya St., Moscow, 119991,

8, Orekhovy Boulevard, Moscow, 115682

 

serg_avdeev@list.ruПервый Московский государственный медицинский университет имени И.М. Сеченова (Сеченовский Университет); Научно-исследовательский институт пульмонологииРоссияSechenov First Moscow State Medical University (Sechenov University); Research Institute for Pulmonology of the Federal Medical Biological AgencyRussian FederationПервый Московский государственный медицинский университет имени И.М. Сеченова (Сеченовский Университет)РоссияSechenov First Moscow State Medical University (Sechenov University)Russian Federation20252206202509107117Copyright © Трушенко Н.В., Левина Ю.А., Лавгинова Б.Б., Суворова О.А., Авдеев С.Н., 20252025Трушенко Н.В., Левина Ю.А., Лавгинова Б.Б., Суворова О.А., Авдеев С.Н.Trushenko N.V., Levina Y.A., Lavginova B.B., Suvorova O.А., Avdeev S.N.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/9200

Идиопатический легочный фиброз (ИЛФ) и прогрессирующие легочные фиброзы (ПЛФ) характеризуются неуклонным прогрессированием, крайне неблагоприятным прогнозом и высокой летальностью. Антифибротические препараты, такие как пирфенидон и нинтеданиб, замедляют снижение легочной функции, улучшают выживаемость пациентов и являются основой в лечении ИЛФ. В последние годы активно изучается применение антифибротических препаратов при ПЛФ. Пирфенидон, обладающий антифибротическим, противовоспалительным и антиоксидантным действием, ингибирует ключевые пути фиброгенеза, включая ТФР-β. В данном обзоре представлены современные данные о применении пирфенидона при ИЛФ и ПЛФ, в т. ч. при гиперчувствительном пневмоните, силикозе, интерстициальных заболеваниях легких, ассоциированных с болезнями соединительной ткани. В работе рассмотрены механизмы действия пирфенидона, его доказательная база (включая результаты ключевых клинических исследований CAPACITY, ASCEND и RELIEF), а также данные реальной клинической практики, подтверждающие, что пирфенидон замедляет прогрессирование заболевания и снижение легочных функциональных показателей как при ИЛФ, так и ПЛФ. В сравнительной оценке пирфенидона и нинтеданиба оба препарата демонстрируют сопоставимую эффективность, однако пирфенидон имеет более предсказуемый профиль безопасности с дозозависимыми желудочно-кишечными нарушениями и фотосенсибилизацией в качестве основных нежелательных явлений. Серьезные побочные эффекты наблюдаются редко, что позволяет рассматривать пирфенидон как относительно безопасный вариант терапии. Особое внимание уделено новым стратегиям терапии, таким как комбинированное применение с нинтеданибом, использование низких доз и ингаляционных форм препарата. Приводятся рекомендации по оптимизации лечения с учетом профиля безопасности пирфенидона и индивидуальных особенностей пациентов. Обсуждаются перспективы дальнейшего использования пирфенидона при различных вариантах ПЛФ.

Both idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) are characterized by steady progression, dismal prognosis and high mortality. Antifibrotic therapies such as pirfenidone and nintedanib slow the decline in lung function and improve patient survival, serving as the cornerstone of treatment for IPF. The use of antifibrotics in PPF has been actively explored in recent years. Pirfenidone, with its antifibrotic, anti-inflammatory, and antioxidant properties, inhibits key pathways of fibrogenesis, including TGF-β-mediated mechanisms. This review presents current data on the use of pirfenidone in IPF and PPF, including hypersensitivity pneumonitis, silicosis, and interstitial lung disease associated with connective tissue diseases. The paper reviews the mechanisms of action of pirfenidone, its evidence base (including the results of the pivotal CAPACITY, ASCEND and RELIEF clinical trials) as well as data from real-world clinical practice confirming that pirfenidone slows disease progression and decline in pulmonary function parameters in both IPF and PPF. In a comparative evaluation of pirfenidone and nintedanib, both drugs demonstrate comparable efficacy, but pirfenidone has a more predictable safety profile, with dose-related gastrointestinal disturbances and photosensitization as the most common adverse events. Serious side effects are rarely observed, making pirfenidone a relatively safe treatment option. New therapeutic strategies such as combination with nintedanib, low dose and inhaled forms of the drug are emphasized. Special attention is given to treatment optimization, considering pirfenidone’s safety profile and individual patient characteristics. The prospects for further applications of pirfenidone across different PPF subtypes are discussed.

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