Modern Pediatrics.Ukraine.2020.1(105):49-62; doi 10.15574/SP.2020.105.49
Охотникова Е. Н., Кваченюк Е. Г., Меллина К. В., Поночевная Е. В.
Национальная медицинская академия последипломного образования имени П.Л. Шупика, г. Киев, Украина

Для цитирования: Охотникова ЕН, Кваченюк ЕГ, Меллина КВ, Поночевная ЕВ. (2020). Эозинофильный гранулематоз с полиангиитом: современный взгляд на патогенез и лечение (литературный обзор). Современная педиатрия. Украина. 1(105): 49-62; doi 10.15574/SP.2020.105.51
Статья поступила в редакцию 30.11.2019 г., принята в печать 06.02.2020 г.

Эозинофильный гранулематоз с полиангиитом (ЭГПА), ранее известный как синдром Чарджа—Стросса, представляет собой некротизирующий васкулит сосудов малого и среднего калибра и характеризуется такими клиническими проявлениями, как астма, гиперэозинофилия крови, тканей и внелегочные симптомы. Несмотря на то, что ЭГПА встречается достаточно редко, в последние годы данное заболевание является объектом прицельного изучения. Это обусловлено тем, что ЭГПА — концептуально сложная патология, которая имеет двойную категоризацию с системными васкулитами (СВ) и гиперэозинофильными синдромами (ГЭС). В настоящее время остаются открытыми вопросы патофизиологической и диагностической неопределенности заболевания, так как до сих пор не до конца изучено взаимодействие между эозинофилами и антинейтрофильными цитоплазматическими антителами (АНЦА), которые являются особенностями патогенеза данного васкулита. Результаты последних исследований указывают на фенотипические подтипы ЭГПА на основании АНЦА-статуса, однако на сегодня недостаточно имеющейся информации о методах лечения с учетом данных фенотипов болезни. ЭГПА также остается диагностической проблемой, потому что в течение длительного времени может рассматриваться только в плоскости аллергической патологии. Астма может быть основным проявлением болезни, а хроническая потребность в приеме глюкокортикостероидов может маскировать другие признаки заболевания. Активное изучение иммунной дисрегуляции и генетической предрасположенности организма пациентов с ЭГПА может решить проблему классификации с уточнением подтипов болезни и дать возможность вовремя диагностировать данное заболевание, а также использовать специально адаптированные методы лечения.
Ключевые слова: эозинофильный гранулематоз с полиангиитом, синдром Чарджа—Стросса, гиперэозинофильный синдром, бронхиальная астма.
Авторы заявляют об отсутствии конфликта интересов.

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