medsovetМедицинский СоветMeditsinskiy sovet = Medical Council2079-701X2658-5790REMEDIUM GROUP Ltd.10.21518/2079-701X-2020-17-99-106medsovet-5882Research ArticleИнтерстициальные болезни легкихInterstitial lung diseaseИнтерстициальные заболевания легких с прогрессирующим легочным фиброзом: патогенетические особенности и подходы к терапииInterstitial lung diseases with progressive pulmonary fibrosis: pathogenetic features and approaches to therapyhttps://orcid.org/0000-0002-1166-9717KузубовaН. А.KuzubovaN. A.

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

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

Natalia A. Kuzubova, Dr. of Sci. (Med.), Deputy Director of Research Institute of Pulmonology

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

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

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

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

Olga N. Titova, Dr. of Sci. (Med.), Professor, Director of Research Institute of Pulmonology

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

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

Склярова Дарья Борисовна, кандидат медицинских наук, врач-пульмонолог

191180, Санкт-Петербург, Лазаретный переулок, д. 4

Daria B. Skliarova, Cand. of Sci. (Med.), Pulmonologist

4, Lazaretny Lane, St Petersburg, 191180

darya_sklyarova@mail.ruПервый Санкт-Петербургский государственный медицинский университет им. академика И.П. ПавловаРоссияPavlov First Saint Petersburg State Medical UniversityRussian FederationВведенская городская клиническая больницаРоссияVvedenskaya City Clinical HospitalRussian Federation20202111202001799106Copyright © Kузубовa Н.А., Tитовa О.Н., Склярова Д.Б., 20202020Kузубовa Н.А., Tитовa О.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/5882

 У ряда пациентов с интерстициальными заболеваниями легких (ИЗЛ) различной этиологии, включающими в себя гиперсенситивный пневмонит, диффузные болезни соединительной ткани (ревматоидный артрит, системная склеродермия, дерматомиозит), саркоидоз, идиопатическую неспецифическую интерстициальную пневмонию (НСИП) и неклассифицируемые ИЗЛ, развивается стремительное ухудшение вентиляционной функции легких, обусловленное прогрессированием фибротических изменений, сопровождающееся снижением физической работоспособности и качества жизни. Предложено выделение прогрессирующего фибротического фенотипа среди ИЗЛ, сходных по патогенетическим механизмам, лучевому паттерну, клиническому течению и прогнозу. Прогрессирующее течение фибротического процесса оценивается по снижению форсированной жизненной емкости легких (ФЖЕЛ), увеличению выраженности признаков легочного фиброза по данным компьютерной томографии (КТ) и ухудшению респираторных симптомов. Выделяют несколько факторов риска прогрессирования ИЗЛ, такие как мужской пол, пожилой возраст, более низкая исходная легочная функция и рентгенологическая или патологическая картина обычной интерстициальной пневмонии (ОИП). В настоящее время активно изучается роль антифибротических препаратов в лечении этой патологии. Ранее общепринятым подходом было использование этой группы препаратов у пациентов с идиопатическим легочным фиброзом (ИЛФ) и иммуносупрессивных препаратов у пациентов с другими фиброзными подтипами ИЗЛ. Однако результаты клинических исследований показали благоприятный ответ на антифибротическую терапию при более широком спектре фибротических ИЗЛ, проявляющийся в уменьшении ежегодной скорости снижения ФЖЕЛ. И в 2020 г. было одобрено использование первого антифибротического препарата для лечения больных ИЗЛ с прогрессирующим легочным фиброзом, не относящимся к идиопатическому легочному фиброзу (ИЛФ).

A number of patients with interstitial lung diseases (ILD) of various etiologies, including hypersensitive pneumonitis, diffuse connective tissue diseases (rheumatoid arthritis, systemic scleroderma, dermatomyositis), sarcoidosis, idiopathic non-specific interstitial pneumonia (NSIP) and unclassified ILD develop rapid deterioration of lung ventilation function due to the progression of fibrotic changes, accompanied by a decrease in physical performance and quality of life. It is proposed to distinguish a progressive fibrotic phenotype from those with similar pathogenetic mechanisms, radiologic pattern, clinical course, and prognosis. The progressive course of the fibrotic process is assessed by reducing the forced vital capacity of the lungs (FVC), increasing the severity of signs of pulmonary fibrosis according to computed tomography (CT) and worsening respiratory symptoms. There are several risk factors for the progression of ILD, such as male gender, older age, lower initial pulmonary function, and radiological or pathological picture of usual interstitial pneumonia (UIP). Currently, the role of antifibrotic drugs in the treatment of this pathology is being actively studied. Previously, the common approach was to use this group of drugs in patients with idiopathic pulmonary fibrosis (IPF) and immunosuppressive drugs in patients with other fibrotic subtypes of IL. However, the results of clinical studies have shown a favorable response to antifibrotic therapy for a wider range of fibrotic ILD, manifested in a decrease in the annual rate of FVC reduction. And in 2020, the use of the first anti-fibrotic drug was approved for the treatment of patients with advanced pulmonary fibrosis, NOT related to idiopathic pulmonary fibrosis (IPF).

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