pulmoПульмонологияPULMONOLOGIYA0869-01892541-9617Scientific and Practical Journal “PULMONOLOGIYA” LLC10.18093/0869-0189-2017-27-1-56-64pulmo-819Research ArticleОБЗОРЫREVIEWСовременные представления об идиопатическом легочном фиброзе: в фокусе – биомаркерыCurrent concepts of idiopathic pulmonary fibrosis: focus on biomarkersАнаевЭ. Х.AnaevE. Kh.

д. м. н., заведующий лабораторией неинвазивных методов диагностики клинического отдела 

Doctor of Medicine, head of Laboratory of Non-invasive Diagnostic Methods, Clinical Division

el_anaev@hotmail.comФедеральное государственное бюджетное учреждение «Научно-исследовательский институт пульмонологии Федерального медико-биологического агентства России»РоссияFederal Pulmonology Research Institute, Federal Medical and Biological Agency of RussiaRussian Federation2017020520172715664Copyright © Анаев Э.Х., 20172017Анаев Э.Х.Anaev E.K.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/819

Идиопатический легочный фиброз (ИЛФ) – хроническое прогрессирующее заболевание легких с неблагоприятным прогнозом. В последние годы благодаря разработке международных диагностических критериев, основой которых явились клинические, физиологические, рентгенологические и морфологические особенности, диагностика ИЛФ улучшилась. Тем не менее имеющиеся в настоящее время клинические данные не позволяют точно прогнозировать болезнь, прогрессирование которой может протекать непредсказуемо – как медленно, так и быстро. В 5 % случаев ИЛФ протекает с обострением. Указанными проблемами обусловлена необходимость разработки и валидации не только диагностических биомаркеров, специфичных для ИЛФ, но и прогностических биомаркеров течения болезни для принятия тактики лечения, в т. ч. трансплантации легких. После регистрации противофиброзных препаратов (пирфенидона и нинтеданиба) и выявления новых потенциальных терапевтических мишеней появилась необходимость в поиске терагностических маркеров – биомаркеров, способных оценить эффективность препарата в начале лечения, что позволит избежать нежелательных явлений и повысить эффективность лечения. Кроме того, с помощью имеющихся в настоящее время методов не представляется возможным выявить ИЛФ в ранней стадии, прогнозировать течение болезни, а также оценить ответ на противофиброзную терапию. Благодаря последним достижениям в понимании нескольких взаимосвязанных патогенетических путей, лежащих в основе ИЛФ, выявлены различные молекулярные фенотипы в результате сложного взаимодействия генетических, эпигенетических, транскрипционных, метаболических факторов, а также факторов окружающей среды. Для более точной и ранней диагностики и улучшения прогнозирования течения ИЛФ необходима разработка и обоснование диагностических и прогностических биомаркеров.

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease with a poor prognosis. In recent years, the development of international diagnostic criteria based on clinical, physiological, radiological, and histopathological appearances, improved diagnosis of IPF. However, currently available clinical data do not accurately predict the course of the disease, which can vary from a slowly progressing to rapidly progressive, and, in 5% of cases, is punctuated by episodes of rapid acute exacerbation. These challenges highlight the need for the development and validation of diagnostic biomarkers specific to IPF and prognostic biomarkers of future disease behavior to guide treatment decisions, including referral for transplant. The recent approval of pirfenidone and nintedanib and the identification of new potential therapeutic targets have created an urgent need for theragnostic markers, i.e. biomarkers able to assess, ideally at an early stage, therapeutic response to a given drug. This will avoid the side effects and increase efficacy of treatment. In addition, the currently available methods are not able to identify the IPF in the early stage to predict the course of disease, and to assess response to antifibrotic therapy. Recent advances in understanding the multiple interrelated pathogenic pathways underlying IPF have identified various molecular phenotypes resulting from complex interactions among genetic, epigenetic, transcriptional, post-transcriptional, metabolic, and environmental factors. The development and validation of diagnostic and prognostic biomarkers are necessary to enable a more precise and earlier diagnosis of IPF and to improve prediction of future disease behavior.

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