medsovetМедицинский СоветMeditsinskiy sovet = Medical Council2079-701X2658-5790REMEDIUM GROUP Ltd.10.21518/ms2023-013medsovet-7435Research ArticleИНТЕРСТИЦИАЛЬНЫЕ ЗАБОЛЕВАНИЯ ЛЕГКИХINTERSTITIAL PULMONARY DISEASESПрогностические биомаркеры прогрессирующего легочного фиброза у пациентов с интерстициальными заболеваниями легкихPrognostic biomarkers of progressive pulmonary fibrosis in patients with interstitial lung diseaseshttps://orcid.org/0000-0002-1166-9717KузубовaН. А.KuzubovaN. A.

Кузубова Наталия Анатольевна – доктор медицинских наук, заместитель директора по научной работе Научно-исследовательского института пульмонологии Научно-клинического исследовательского центра.

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

Natalia A. Kuzubova - Dr. Sci. (Med.), Deputy Director for Research, Research Institute of Pulmonology, Scientific and Clinical Research Centre, Pavlov First Saint Petersburg State Medical University.

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

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

Титова Ольга Николаевна - доктор медицинских наук, профессор, директор Научно-исследовательского института пульмонологии Научно-клинического исследовательского центра.

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

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

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

https://orcid.org/0000-0001-7052-6903СкляроваД. Б.SkliarovaD. B.

Склярова Дарья Борисовна - кандидат медицинских наук, старший научный сотрудник Научно-исследовательского института пульмонологии Научно-клинического исследовательского центра.

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

Daria B. Skliarova - Cand. Sci. (Med.), Senior Research Associate, Scientific and Clinical Research Centre, Pavlov First Saint Petersburg State Medical University.

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

darya_sklyarova@mail.ruПервый Санкт-Петербургский государственный медицинский университет имени академика И.П. ПавловаРоссияPavlov First Saint Petersburg State Medical UniversityRussian Federation202313042023048691Copyright © Kузубовa Н.А., Tитовa O.Н., Склярова Д.Б., 20232023Kузубовa Н.А., Tитовa O.Н., Склярова Д.Б.Kuzubova N.A., Titova O.N., Skliarova D.B.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/7435

У высокой доли пациентов с различными интерстициальными заболеваниями легких (ИЗЛ) развивается прогрессирующий легочный фиброз (ПЛФ), связанный с ухудшением качества жизни, снижением функционального статуса и ранней смертностью. ПЛФ диагностируется у пациентов с ИЗЛ известной или неизвестной этиологии, отличной от идиопатического легочного фиброза (ИЛФ), у которых есть рентгенологические признаки легочного фиброза и по крайней мере два из трех критериев прогрессирования, включающих в себя клинико-функциональные и рентгенологические показатели, которые возникли в течение последнего года при отсутствии альтернативных причин ухудшения. Подтипы ИЗЛ, которые подвержены риску развития прогрессирующего фиброзирующего фенотипа, включают ИЗЛ, связанные с заболеваниями соединительной ткани, такими как ревматоидный артрит и системная склеродермия; саркоидозом; гиперчувствительным пневмонитом, профессиональными заболеваниями легких. В 2022 г. появились новые критерии диагностики ПЛФ. Легочный фиброз инициируется микроповреждениями альвеолярного эпителия различной этиологии, в дальнейшем его прогрессирование включает в себя сходные патофизиологические механизмы, что позволяет предположить наличие единых биомаркеров активности заболевания для широкого спектра ИЗЛ. Биомаркеры могут помочь не только выявить пациентов с риском прогрессирования, но и контролировать ранний ответ на лечение. Создание национальных и международных регистров позволяет проводить длительный мониторинг пациентов с ПФЛ, ответить на вопросы о долгосрочной эффективности лечения. На сегодняшний день обсуждается ряд молекулярных факторов, претендующих на роль предикторов, позволяющих верифицировать диагноз, определить прогноз заболевания и ответ на терапию.

A high proportion of patients with various interstitial lung diseases (ILD) develop progressive pulmonary fibrosis (PLF) associated with a deterioration in the quality of life, a decrease in functional status and early mortality. PLF is diagnosed in patients with ILD of known or unknown etiology other than idiopathic pulmonary fibrosis (ILF), who have radiological signs of pulmonary fibrosis, and at least two of the three criteria for progression, including clinical, functional and radiological indicators that have arisen over the past year in the absence of alternative causes of deterioration. Subtypes of ILD that are at risk of developing a progressive fibrosing phenotype include ILD associated with connective tissue diseases, such as rheumatoid arthritis and systemic scleroderma; sarcoidosis; hypersensitive pneumonitis, occupational lung diseases. In 2022, new criteria for the diagnosis of PLF appeared. The existing evidence of common pathogenesis mechanisms leading to progressive pulmonary fibrosis suggests the presence of uniform biomarkers of disease activity for a wide range of diseases. Pulmonary fibrosis is initiated by microinjuries of the alveolar epithelium of various etiologies, in the future its progression includes similar pathophysiological mechanisms, which suggests the presence of uniform biomarkers of disease activity for a wide range of diseases. Biomarkers can help not only identify patients at risk of progression, but also allow monitoring the early response to treatment. Biomarker research to date has identified a number of molecular markers that predict the presence of the disease, prognosis and/or response to treatment. The creation of national and international registries allows for long-term monitoring of patients with PLF, to answer questions about the long-term effectiveness of treatment. To date, a number of molecular factors that claim to be predictors are being discussed, allowing to verify the diagnosis, determine the prognosis and response to therapy.

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