Идиопатический легочный фиброз и гиперсенситивный пневмонит: новый взгляд на роль генетических и эпигенетических факторов в развитии и течении заболеваний

   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.

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