References

avk

Архивъ внутренней медицины

The Russian Archives of Internal Medicine

2226-67042411-6564

“SINAPS” LLC

10.20514/2226-6704-2022-12-4-267-275

avk-1479

Research Article

ОБЗОРНЫЕ СТАТЬИ

REVIEW ARTICLES

Перспективы лечения идиопатического легочного фиброза

Prospects for Treatment of Idiopathic Pulmonary Fibrosis

Мустафин

Р. Н.

Mustafin

R. N.

Уфа

Ufa

ФГБОУ ВО «Башкирский государственный медицинский университет»РоссияBashkir State Medical UniversityRussian Federation

2022

30072022

124267275

2022

Мустафин Р.Н.

Mustafin R.N.

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/1479

Идиопатический легочный фиброз (ИЛФ) является тяжелым прогрессирующим заболеванием легких неизвестной этиологии со средней распространенность ю 15 на 100000 населения в мире. Различают спорадические, синдромальные и семейные случаи болезни. Спорадические случаи относятся к многофакторным заболеваниям и ассоциированы с возрастом, вирусными инфекциями, курением и вдыханием пыли, контактом с химическими реагентами и лекарствами, гастроэзофагальной рефлюксной болезнью. Выявлена ассоциация спорадического ИЛФ с аллельными вариантами генов AKAP13, ATP11A, DPP9, DSP, IVD, IL1RN, FAM13A, MUC5B, SFTPC, SPPL2C, TERC, TERT, TOLLIP. Синдромальный ИЛФ описан при синдроме Германского-Пудлака. Семейные случаи болезни обусловлены мутациями в генах, кодирующих белки сурфактанта (SFTPC), муцина (MUC5B), нуклеазу деаденилирования (PARN), участвующие в функционировании теломер (RTEL1, TERC, TERT). В 2000 году Американское торакальное сообщество рекомендовало глюкокортикоиды и цитостатики для лечения ИЛФ с целью воздействия на воспалительный процесс при активации фибробластов и их аккумулировании во внеклеточном матриксе легких. Эти рекомендации до сих пор используются практике, несмотря на публикации достоверных данных о повышенной смертности и случаев госпитализации пациентов с ИЛФ, принимающих преднизолон и азатиоприн. Согласно данным недавних метаанализов, наиболее эффективными в лечении ИЛФ являются пирфенидон (ингибитор синтеза факторов роста проколлагенов I и II) и нинтенадиб (ингибитор тирозинкиназы). Поскольку важную роль в этиопатогенезе болезни играют генетические факторы, перспективен поиск методов таргетной терапии с использованием в качестве мишеней специфических некодирующих РНК, изменения экспрессии которых не характерны для других бронхолегочных заболеваний. К ним относятся miR-9-5p, miR-27b, miR-153, miR-184, miR-326, miR-374, miR-489, miR-630, miR-1343 (уровень их снижается при болезни); miR-340, miR-424, miR-487b, miR-493, lncRNA AP003419.16, lncRNA AP003419.16 (повышенная экспрессия при ИЛФ).

Idiopathic pulmonary fibrosis (IPF) is a severe, progressive lung disease of unknown etiology with an average worldwide prevalence of 15 per 100,000. According to the etiology, IPF is classified into sporadic, syndromic, and familial cases. Sporadic cases refer to multifactorial diseases and are associated with age, viral infections, smoking and inhalation of dust, contact with chemicals and drugs, gastroesophageal reflux disease. There were revealed an association of sporadic IPF with allelic variants of the genes AKAP13, ATP11A, DPP9, DSP, IVD, IL1RN, FAM13A, MUC5B, SFTPC, SPPL2C, TERC, TERT, TOLLIP. Syndromal IPF develops in German-Pudlak syndrome. Familial cases of the disease are caused by mutations in the genes encoding surfactant (SFTPC), mucin (MUC5B), deadenylation nuclease (PARN), components of telomere functioning (RTEL1, TERC, TERT). In 2000, the American Thoracic Society recommended glucocorticoids and cytostatics for the treatment of ELISA in order to influence the inflammatory process due to the activation of fibroblasts and their accumulation in the extracellular matrix of the lungs. These recommendations are still used by many doctors, despite the publication of reliable data on the increased mortality and hospitalizations of IPF patients taking prednisolone and azathioprine. According to recent meta-analyzes, pirfenidone (an inhibitor of the synthesis of procollagen I and II growth factors) and nintenadib (a tyrosine kinase inhibitor) are the most effective treatments for IPF. Since genetic factors play an important role in the etiopathogenesis of the disease, it is promising to search for methods of targeted therapy for IPF using specific noncoding RNAs as targets, changes in the expression of which are not specific of other bronchopulmonary diseases. These RNAs include miR-9-5p, miR-27b, miR-153, miR-184, miR-326, miR-374, miR-489, miR-630, miR-1343 (decreased expression in IPF); miR-340, miR-424, miR-487b, miR-493, lncRNA AP003419.16, lncRNA AP003419.16 (increased expression in IPF).

диагностикаидиопатический легочный фиброзлечениемеханизм развитиямикроРНКнаследственность

diagnosisidiopathic pulmonary fibrosistreatmentdevelopmental mechanismmicroRNAheredity

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