pulmoПульмонологияPULMONOLOGIYA0869-01892541-9617Scientific and Practical Journal “PULMONOLOGIYA” LLC10.18093/0869-0189-2022-32-3-473-495pulmo-4111Research ArticleКЛИНИЧЕСКИЕ РЕКОМЕНДАЦИИCLINICAL GUIDELINESИдиопатический легочный фиброз: федеральные клинические рекомендации по диагностике и лечениюFederal clinical guidelines on diagnosis and treatment of idiopathic pulmonary fibrosishttps://orcid.org/0000-0002-5999-2150АвдеевС. Н.AvdeevS. N.

д. м. н., профессор, академик Российской академии наук, заведующий кафедрой пульмонологии Института клинической медицины имени Н.В.Склифосовского, 119991, Москва, ул. Трубецкая, 8, стр. 2;

ведущий научный сотрудник, 115682, Москва, Ореховый бульвар, 28

Doctor of Medicine, Professor, Academician of Russian Academy of Sciences, Head of the Department of Pulmonology, N.V.Sklifosovsky Institute of Clinical Medicine, ul. Trubetskaya 8, build. 2, Moscow, 119991;

Leading Researcher, Orehovyy bul’var 28, Moscow, 115682

serg_avdeev@list.ruhttps://orcid.org/0000-0002-4044-674XАйсановЗ. Р.AisanovZ. R.

д. м. н., профессор кафедры пульмонологии, 

117997, Москва, ул. Островитянова, 1

Doctor of Medicine, Professor, Department of Pulmonology, 

ul. Ostrovityanova 1, Moscow, 117997

aisanov@mail.ruhttps://orcid.org/0000-0001-6050-724XБелевскийА. С.BelevskiyA. S.

д. м. н., профессор, заведующий кафедрой пульмонологии, 

117997, Москва, ул. Островитянова, 1

Doctor of Medicine, Professor, Head of Department of Pulmonology, Faculty of Postgraduate Physician Training, 

ul. Ostrovityanova 1, Moscow, 117997

pulmobas@yandex.ruhttps://orcid.org/0000-0001-5191-445XИльковичМ. М.IlkovichM. M.

д. м. н., профессор, заведующий кафедрой пульмонологии факультета последипломного образования, директор Научно-исследовательского института интерстициальных и орфанных заболеваний легких,

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

Doctor of Medicine, Professor, Head of pulmonology Department of Postgraduate School, Director of Interstitial and Orphan Lung Diseases Research Institute, 

ul. L’va Tolstogo 6–8, Saint-Petersburg, 197089

mih.ilkovich@yandex.ruКоганЕ. А.KoganE. A.

д. м. н., профессор, заведующая кафедрой патологической анатомии имени А.И.Струкова,

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

Doctor of Medicine, Professor, Head of A.I.Strukov Department of Pathological Anatomy, 

ul. Trubetskaya 8, build. 2, Moscow, 119991

koganevg@gmail.comhttps://orcid.org/0000-0002-3174-5000МержоеваЗ. М.MerzhoevaZ. M.

к. м. н., доцент кафедры пульмонологии, заведующая пульмонологическим отделением Университетской клинической больницы № 4,

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

Candidate of Medicine, Associate Professor, Pulmonology Department, Head of Pulmonology Department, University Clinical Hospital No4, 

ul. Trubetskaya 8, build. 2, Moscow, 119991

zamira.merzoeva@bk.ruПетровД. В.PetrovD. V.

врач-пульмонолог,

644112, Омск, ул. Перелета, 7

pulmonologist, 

ul. Pereleta 7, Omsk, 644112

dmitrpetrov@bk.ruhttps://orcid.org/0000-0001-8170-1260СамсоноваМ. В.SamsonovaM. V.

д. м. н., заведующая лабораторией патологической анатомии и иммунологии,

115682, Москва, Ореховый бульвар, 28

Doctor of Medicine, Head of Laboratory of Pathological Anatomy and Immunology,

Orehovyy bul’var 28, Moscow, 115682

samary@mail.ruТерпигоревС. А.TerpigorevS. A.

д. м. н.; руководитель отделения профессиональной патологии и врачебно-трудовой экспертизы,

129110, Москва, ул. Щепкина, 61 / 2, корп. 1

Doctor of Medicine, Head of Department of Occupational Diseases and Medical Expertise,

ul. Shchepkina 61/2, Moscow, 129110

smith42@yandex.ruhttps://orcid.org/0000-0002-0685-4133ТрушенкоН. В.TrushenkoN. V.

к. м. н., ассистент кафедры пульмонологии, 119991, Москва, ул. Трубецкая, 8, стр. 2;

научный сотрудник, 115682, Москва, Ореховый бульвар, 28

Candidate of Medicine; Assistant Lecturer, Department of Pulmonology, ul. Trubetskaya 8, build. 2, Moscow, 119991;

Researcher, Orehovyy bul’var 28, Moscow, 115682

trushenko.natalia@yandex.ruhttps://orcid.org/0000-0003-2742-3794ТрофименкоИ. Н.TrofimenkoI. N.

д. м. н., заведующая кафедрой клинической аллергологии и пульмонологии,

664049, Иркутск, мкр Юбилейный, 100

Doctor of Medicine, Head of the Department of Clinical Allergology and Pulmonology, 

mkr Yubilejnyy 100, Irkutsk, 664079

tin11@mail.ruhttps://orcid.org/0000-0003-3931-1431ТюринИ. Е.TyurinI. E.

д. м. н., профессор, заведующий кафедрой рентгенологии и радиологии,

123995, Москва, ул. Баррикадная, 2, стр. 1

Doctor of Medicine, Professor, Head of Department of Radiology, 

 ul. Barrikadnaya 2/1, Moscow, 123995

igortyurin@gmail.comhttps://orcid.org/0000-0002-0158-7056ЧерняевА. Л.ChernyaevA. L.

д. м. н., профессор, заведующий отделом фундаментальной пульмонологии, 115682, Москва, Ореховый бульвар, 28;

профессор кафедры патологической анатомии и клинической патологической анатомии лечебного факультета, 117997, Москва, ул. Островитянова, 1;

ведущий научный сотрудник лаборатории клинической морфологии, 117418, Москва, ул. Цюрупы, 3

 

Doctor of Medicine, Head of the Department of Fundamental Pulmonology, Orehovyy bul’var 28, Moscow, 115682;

Professor, Department of Pathological Anatomy and Clinical Pathological Anatomy, Faculty of Medicine, ul. Ostrovityanova 1, Moscow, 117997;

Leading Researcher, Laboratory of Clinical Morphology, ul. Tsyurupy 3, Moscow, 117418

cheral12@gmail.comhttps://orcid.org/0000-0002-5902-6198ЧернякБ. А.ChernyakB. A.

д. м. н., профессор кафедры клинической аллергологии и пульмонологии,

664049, Иркутск, мкр Юбилейный, 100

Doctor of Medicine, Professor, Department of Clinical Allergology and Pulmonology, 

mkr Yubileynyy 100, Irkutsk, 664079

ba.chernyak@gmail.comhttps://orcid.org/0000-0002-2001-5504ЧернякА. В.ChernyakA. V.

к. м. н., заведующий лабораторией функциональных и ультразвуковых методов исследования,

115682, Москва, Ореховый бульвар, 28

Candidate of Medicine, Head of Laboratory of Functional and Ultra-sound Investigations,

Orehovyy bul’var 28, Moscow, 115682

achi2000@mail.ruhttps://orcid.org/0000-0002-5536-9388ЧикинаС. Ю.ChikinaS. Yu.

к. м. н., ассистент кафедры пульмонологии Института клинической медицины имени Н.В.Склифосовского,

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

Candidate of Medicine, Assistant Lecturer, Department of Pulmonology, N.V.Sklifosovskiy Institute of Clinical Medicine, 

ul. Trubetskaya 8, build. 2, Moscow, 119991

svch@list.ruhttps://orcid.org/0000-0002-6808-5528ЧучалинА. Г.ChuchalinA. G.

д. м. н., профессор, академик Российской академии наук, заведующий кафедрой госпитальной терапии педиатрического факультета, 117997, Москва, ул. Островитянова, 1;

председатель правления Российского респираторного общества

Doctor of Medicine, Professor, Academician of Russian Academy of Sciences, Head of Department of Hospital Internal Medicine, Pediatric Faculty, 

ul. Ostrovityanova 1, Moscow, 117997

pulmomoskva@mail.ruhttps://orcid.org/0000-0002-1908-5601ШмелевЕ. И.ShmelevE. I.

д. м. н., профессор, заведующий отделом дифференциальной диагностики туберкулеза легких и экстракорпоральных методов лечения,

107564, Москва, Яузская аллея, 2

Doctor of Medicine, Professor, Head of Differential Diagnosis of Pulmonary Tuberculosis and Extracorporeal Methods of Treatment Department, 

Yauzskaya alleya 2, Moscow, 107564

eishmelev@mail.ruФедеральное государственное автономное образовательное учреждение высшего образования «Первый Московский государственный медицинский университет имени И.М.Сеченова» Министерства здравоохранения Российской Федерации (Сеченовский Университет); Федеральное государственное бюджетное учреждение «Научно-исследовательский институт пульмонологии» Федерального медико-биологического агентстваРоссияFederal State Autonomous Educational Institution of Higher Education I.M.Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University); Federal Pulmonology Research Institute, Federal Medical and Biological Agency of RussiaRussian FederationФедеральное государственное бюджетное образовательное учреждение высшего образования «Российский национальный исследовательский медицинский университет имени Н.И.Пирогова» Министерства здравоохранения Российской ФедерацииРоссияPirogov Russian National Research Medical University (Pirogov Medical University), Ministry of Health of the Russian FederationRussian FederationФедеральное государственное бюджетное образовательное учреждение высшего образования «Первый Санкт-Петербургский государственный медицинский университет имени академика И.П.Павлова» Министерства здравоохранения Российской ФедерацииРоссияFederal State Budgetary Educational Institution of Higher Education “Academician I.P. Pavlov First St. Petersburg State Medical University” of the Ministry of Healthcare of Russian FederationRussian FederationФедеральное государственное автономное образовательное учреждение высшего образования «Первый Московский государственный медицинский университет имени И.М.Сеченова» Министерства здравоохранения Российской Федерации (Сеченовский Университет)РоссияFederal State Autonomous Educational Institution of Higher Education I.M.Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University)Russian FederationБюджетное учреждение здравоохранения Омской области «Городская клиническая больница № 1 имени Кабанова А.Н.»РоссияBudgetary Healthcare Institution, Omsk region “City Clinical Hospital No.1 named after Kabanov A.N.”Russian FederationФедеральное государственное бюджетное учреждение «Научно-исследовательский институт пульмонологии» Федерального медико-биологического агентстваРоссияFederal Pulmonology Research Institute, Federal Medical and Biological Agency of RussiaRussian FederationГосударственное бюджетное учреждение здравоохранения Московской области «Московский областной научно-исследовательский клинический институт им. М.Ф.Владимирского»РоссияM.F.Vladimirskiy State Moscow Regional Research Clinical InstituteRussian FederationИркутская государственная медицинская академия последипломного образования – филиал Федерального государственного бюджетного образовательного учреждения дополнительного профессионального образования «Российская медицинская академия непрерывного профессионального образования» Министерства здравоохранения Российской ФедерацииРоссияIrkutsk State Medical Academy of Postgraduate Education – Branch Campus of the Federal State Budgetary Educational Institution of Further Professional Education “Russian Medical Academy of Continuing Professional Education”, Healthcare Ministry of the Russian FederationRussian FederationФедеральное государственное бюджетное образовательное учреждение дополнительного профессионального образования «Российская медицинская академия непрерывного профессионального образования» Министерства здравоохранения Российской ФедерацииРоссияRussian Federal Academy of Continued Medical Education, Healthcare Ministry of RussiaRussian FederationФедеральное государственное бюджетное учреждение «Научно-исследовательский институт пульмонологии» Федерального медико-биологического агентства; Федеральное государственное бюджетное образовательное учреждение высшего образования «Российский национальный исследовательский медицинский университет имени Н.И.Пирогова» Министерства здравоохранения Российской Федерации; «Научно-исследовательский институт морфологии человека имени академика А.П.Авцына» Федерального государственного бюджетного научного учреждения «Российский научный центр хирургии имени академика Б.В.Петровского»РоссияFederal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia; Pirogov Russian National Research Medical University (Pirogov Medical University), Ministry of Health of the Russian Federation; Avtsyn Research Institute оf Human Morphology оf Federal State Budgetary Scientific Institution “Petrovsky National Research Centre оf Surgery”Russian FederationИркутская государственная медицинская академия последипломного образования – филиал Федерального государственного бюджетного образовательного учреждения дополнительного профессионального образования «Российская медицинская академия непрерывного профессионального образования» Министерства здравоохранения Российской ФедерацииРоссияIrkutsk State Medical Academy of Postgraduate Education – Branch Campus of Irkutsk State Medical Academy of Postgraduate TrainingRussian FederationФедеральное государственное бюджетное научное учреждение «Центральный научно-исследовательский институт туберкулеза»РоссияFederal Central Research Institute of TuberculosisRussian Federation202214072022323Спецвыпуск473495Copyright © Авдеев С.Н., Айсанов З.Р., Белевский А.С., Илькович М.М., Коган Е.А., Мержоева З.М., Петров Д.В., Самсонова М.В., Терпигорев С.А., Трушенко Н.В., Трофименко И.Н., Тюрин И.Е., Черняев А.Л., Черняк Б.А., Черняк А.В., Чикина С.Ю., Чучалин А.Г., Шмелев Е.И., 20222022Авдеев С.Н., Айсанов З.Р., Белевский А.С., Илькович М.М., Коган Е.А., Мержоева З.М., Петров Д.В., Самсонова М.В., Терпигорев С.А., Трушенко Н.В., Трофименко И.Н., Тюрин И.Е., Черняев А.Л., Черняк Б.А., Черняк А.В., Чикина С.Ю., Чучалин А.Г., Шмелев Е.И.Avdeev S.N., Aisanov Z.R., Belevskiy A.S., Ilkovich M.M., Kogan E.A., Merzhoeva Z.M., Petrov D.V., Samsonova M.V., Terpigorev S.A., Trushenko N.V., Trofimenko I.N., Tyurin I.E., Chernyaev A.L., Chernyak B.A., Chernyak A.V., Chikina S.Y., Chuchalin A.G., Shmelev E.I.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.pulmonology.ru/pulm/article/view/4111

Идиопатический легочный фиброз (ИЛФ) представляет собой особую форму хронической фиброзирующей интерстициальной пневмонии с прогрессирующим течением и плохим прогнозом. Распространенность ИЛФ в Российской Федерации составляет около 8–12 случаев на 100 тыс. населения. Основу терапии ИЛФ составляют препараты с антифибротическими свойствами.

Методология

Методология. Целевой аудиторией данных клинических рекомендаций являются врачи-терапевты, врачи общей практики, врачи-пульмонологи, врачи-патологоанатомы, врачи-рентгенологи и врачи по медицинской реабилитации. Каждый тезис-рекомендация по проведению диагностических и лечебных мероприятий оценивается по шкалам уровней достоверности доказательств от 1 до 5 и шкале оценки уровней убедительности рекомендаций – по категориям А, В, С. Клинические рекомендации содержат также комментарии и разъяснения к указанным тезисам-рекомендациям, алгоритмы по диагностике и лечению ИЛФ, справочные материалы.

Заключение

Заключение. Представлены современные сведения об этиологии, патогенезе, клинических проявлениях, диагностике и лечении ИЛФ. Одобрены Научно-практическим советом Министерства здравоохранения Российской Федерации в 2021 г. 

Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic fibrotic interstitial pneumonia with a progressive course and poor prognosis. The prevalence of IPF in the Russian Federation is about 8 – 12 cases per 100,000 population. The basic treatment of IPF is antifibrotic agents.

Methodology

Methodology. The target audience of these clinical recommendations are therapists, general practitioners, pulmonologists, pathologists, radiologists, and medical rehabilitation doctors. Each thesis-recommendation about diagnostic and therapeutic procedures has been scored according to the scale of classes of recommendations from 1 to 5 and A, B, C scale of the levels of evidence. Clinical recommendations also contain comments and explanations to the theses, algorithms for the diagnosis and treatment of idiopathic pulmonary fibrosis, and reference materials.

Conclusion

Conclusion. The presented clinical guidelines cover current information about the etiology and pathogenesis, clinical manifestations, diagnosis, treatment, and prevention of idiopathic pulmonary fibrosis. These guidelines were approved by the Scientific and Practical Council of the Ministry of Health of the Russian Federation in 2021. 

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