Подходы к лечению лимфангиолейомиоматоза лёгких: от радикальной хирургии до молекулярной биологии

Цель. Сбор и систематизация данных о подходах к лечению лимфангиолейомиоматоза (ЛАМ) лёгких, основанных на представлениях о патогенезе заболевания.

Материалы и методы. Проанализировано 1157 статей и монографий (включая дубли), из которых отобраны 70 оригинальных источников. Поиск источников осуществляли в базах PubMed, eliabrary.ru, CyberLeninka. Глубина поиска составила 50 лет — с 1973 г. по август 2023 г. Авторы акцентировали внимание на актуальных публикациях в высокорейтинговых научных журналах.

Результаты. В обзоре представлены подходы к лечению ЛАМ, основанные как на клинических наблюдениях за течением заболевания, так и на экспериментальных данных о вероятном его патогенезе. Собранные данные представлены в хронологическом порядке, начиная с радикальных методов, основанных на представлениях о безусловной связи развития ЛАМ с воздействием женских половых гормонов. Отдельное внимание уделено препаратам из группы ингибиторов mTOR, в том числе их профилю безопасности. Помимо этого, приведены результаты исследований, демонстрирующих новые перспективные методы лекарственной терапии ЛАМ, сочетающие в себе как применение ингибиторов mTOR с другими препаратами, так и основанных на изолированном использовании альтернативных групп медикаментов.

Заключение. Используемые на настоящий момент методы лекарственной терапии и предлагаемые новые методы направлены лишь на лечение уже сформировавшегося заболевания, а проведение эффективной медикаментозной профилактики ЛАМ сейчас представляется практически невозможным ввиду отсутствия полного понимания патогенеза заболевания и, что более важно, — его этиологии. Данный вопрос является наиболее актуальным в определении дальнейших перспектив развития фармакотерапевтических подходов к ЛАМ.

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