avkАрхивъ внутренней медициныThe Russian Archives of Internal Medicine2226-67042411-6564“SINAPS” LLC10.20514/2226-6704-2024-14-6-405-418LYHBFOavk-1876Research ArticleОБЗОРНЫЕ СТАТЬИREVIEW ARTICLESИдиопатический легочный фиброз и гиперсенситивный пневмонит: новый взгляд на роль генетических и эпигенетических факторов в развитии и течении заболеванийIdiopathic Pulmonary Fibrosis and Hypersensitive Pneumonitis: A Fresh View on The Role of Genetic and Epigenetic Factors in The Development and Course of Diseaseshttps://orcid.org/0009-0005-2398-932XАрхангельскаяЕ. Е.ArkhangelskayaE. E.

Елена Евгеньевна Архангельская, к. м. н., заведующий отделением,  доцент

ГУЗ «СГКб № 8»; отделение пульмонологии; кафедра терапии, гастроэнтерологии и пульмонологии

Саратов

Elena E. Arkhangelskaya, PhD, Head of the Department, Associate Professor

State Healthcare Institution “Samara City Clinical Hospital No. 8”; Pulmonology Department; Department of Therapy, Gastroenterology and Pulmonology

Saratov

orlova_lena78@mail.ruhttps://orcid.org/0000-0001-8300-8988ЛяминаС. В.LyaminaS. V.

Светлана Владимировна Лямина, д. м. н., заведующий лабораторией, профессор

научно-исследовательский центр биомедицинских исследований; лаборатория молекулярной патологии пищеварения; кафедра пропедевтики внутренних болезней и гастроэнтерологии

Саратов

Svetlana V. Lyamina, MD, PhD, Head of the Laboratory, Professor

Research Center for Biomedical Research; Laboratory of Molecular Pathology of Digestion; Department of Propaedeutics of Internal Medicine and Gastroenterology

Saratov

svlvs@mail.ruhttps://orcid.org/0000-0002-9835-694XКожевниковаЕ. О.KozhevnikovaE. O.

Екатерина Олеговна Кожевникова, к. б. н., научный сотрудник

научно-исследовательский центр биомедицинских исследований; лаборатория молекулярной патологии пищеварения

Саратов

Ekaterina O. Kozhevnikova, PhD, research associate

Research Center for Biomedical Research; Laboratory of Molecular Pathology of Digestion

Saratov

katena_94@list.ruhttps://orcid.org/0000-0002-5056-4504КозловаИ. В.KozlovaI. V.

Ирина Вадимовна Козлова, д. м. н., заведующий кафедрой, профессор

кафедра терапии, гастроэнтерологии, пульмонологии

Саратов

Irina V. Kozlova, MD, PhD, Head of the Department, Professor

Department of Therapy, Gastroenterology, Pulmonology

Saratov

kozlova@inbox.ruhttps://orcid.org/0000-0001-6186-6746ШаповаловаТ. Г.ShapovalovaT. G.

Татьяна Германовна Шаповалова, д. м. н., профессор

кафедра терапии, гастроэнтерологии, пульмонологии

Саратов

Tatyana G. Shapovalova, MD, Professor

Department of Therapy, Gastroenterology, Pulmonology

Saratov

t.g.shapovalova@gmail.comhttps://orcid.org/0000-0001-8181-8813ЮреневГ. Л.YurenevG. L.

Георгий Леонидович Юренев, д. м. н., профессор

кафедра пропедевтики внутренних болезней и гастроэнтерологии

Саратов

Georgy L. Yurenev, MD, Professor

Department of Propaedeutics of Internal Medicine and Gastroenterology

Saratov

yurenev@list.ruФГБОУ ВО Саратовский государственный медицинский университет имени В.И. Разумовского Минздрава РоссииРоссияSaratov State Medical University named after V.I. Razumovsky of the Ministry of Health of the Russian FederationRussian FederationФГБОУ ВО «Российский университет медицины» Минздрава РоссииРоссияFederal State Budgetary Educational Institution of Higher Education «Russian University of Medicine» of the Ministry of Health of the Russian FederationRussian Federation202426112024146405418Copyright © Архангельская Е.Е., Лямина С.В., Кожевникова Е.О., Козлова И.В., Шаповалова Т.Г., Юренев Г.Л., 20242024Архангельская Е.Е., Лямина С.В., Кожевникова Е.О., Козлова И.В., Шаповалова Т.Г., Юренев Г.Л.Arkhangelskaya E.E., Lyamina S.V., Kozhevnikova E.O., Kozlova I.V., Shapovalova T.G., Yurenev G.L.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.medarhive.ru/jour/article/view/1876

   Учитывая повсеместно прогрессирующий характер и неблагоприятный прогноз, интерстициальные заболевания легких (ИЗЛ), особенно такие часто встречающиеся варианты, как идиопатический легочный фиброз (ИЛФ) и гиперсенситивный пневмонит (ГП), оправданно привлекают значительное внимание клиницистов и ученых по всему миру. В последние годы все большую актуальность приобретает необходимость углубленного изучения клинических и патогенетических особенностей ИЗЛ, совершенствование существующих и разработка новых эффективных персонализированных подходов тактики ведения этой категории пациентов, на основе наиболее перспективных мишеней воздействия, среди которых все более активно рассматриваются генетические и эпигенетические варианты. Авторами проведен нарративный, описательный обзор литературы, направленный на систематизацию данных об основных известных генетических и эпигенетических механизмах, вовлеченных в патогенез и формирование специфических клинических проявлений ИЛФ и ГП. Отдельно выделены мутации в генах, кодирующих теломеразы, синтез факторов фиброгенеза, полиморфизмы генов муцина, сурфактанта легких, основные эпигенетические изменения, вовлеченные в процессы фиброгенеза. Проанализированы перспективы генетических и эпигенетических исследований для новых фармакологических подходов и мониторинга эффекта уже доступных методов лечения. Поиск литературных источников проводился по базам данных Scopus, Web of Science, MedLine, The Cochrane Library, EMBASE, Global Health, CyberLeninka и РИНЦ, по ключевым словам, «интерстициальные заболевания легких», «идиопатический легочный фиброз», «гиперсенситивный пневмонит», «семейный легочный фиброз», «генетический», «эпигенетический», «прецизионная диагностика», «терапия» с глубиной поиска 20 лет.

   Given their ubiquitous progressive nature and unfavorable prognosis, interstitial lung diseases (ILD), especially such common variants as idiopathic pulmonary fibrosis (IPF) and hypersensitivity pneumonitis (HP), rightly attract considerable attention from clinicians and scientists worldwide. In recent years, the need for an in-depth study of the clinical and pathogenetic features of ILD, improvement of existing approaches and development of effective personalized approaches to the management of this category of patients, based on the most promising targets of action, among which genetic and epigenetic variants are increasingly being considered, has become increasingly important. The authors conducted a non-systematic, descriptive review of the literature aimed at systematizing data on the main known genetic and epigenetic mechanisms involved in the pathogenesis and formation of specific clinical manifestations of IPF and HP. Mutations in genes encoding telomerase, synthesis of fibrogenesis factors, polymorphisms of mucin genes, lung surfactant are highlighted separately, and the main epigenetic changes involved in fibrogenesis processes are highlighted separately. Prospects of genetic and epigenetic studies for new pharmacological approaches and monitoring the effect of already available treatment methods are analyzed. The search for literature sources was conducted in the Scopus, Web of Science, MedLine, The Cochrane Library, EMBASE, Global Health, CyberLeninka, and RSCI databases by the keywords “interstitial lung diseases”, “idiopathic pulmonary fibrosis”, “hypersensitivity pneumonitis”, “familial pulmonary fibrosis”, “genetic”, “epigenetic”, “precision diagnostics”, “therapy” with a search depth of 20 years.

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