Актуальные проблемы склерозирующего холангита у детей

Склерозирующий холангит (СХ) - редкое хроническое заболевание, характеризующееся воспалением и прогрессирующим облитерирующим фиброзом внутрипеченочных и/или внепеченочных желчных протоков. Диагностика основывается на холангиограмме, показывающей расширение желчных протоков, сужение и облитерацию желчного дерева, а также гистологически на наличии воспалительного поражения желчных протоков, приводящего к перидуктальному фиброзу. У детей наиболее распространенный СХ связан с выраженными аутоиммунными проявлениями, перекрывающимися с таковыми при аутоиммунном гепатите (АИГ); эта форма известна как аутоиммунный склерозирующий холангит, АСК. И наоборот, первичный СХ (ПСХ), состояние, при котором термин «первичный» указывает на то, что этиология и патогенез неизвестны, в педиатрии встречается редко. Вторичный СХ (ВСХ) определяет холангиопатию, связанную с идентифицируемой этиологией, такой как иммунодефицит, инфекции или гематологические нарушения. АСХ и ПСХ тесно связаны с воспалительными заболеваниями кишечника (ВЗК). АСХ биохимически хорошо реагирует на иммунодепрессанты и урсодезоксихолевую кислоту (УДХК). Первичные формы лечатся исключительно пероральным применением УДХК, тогда как при вторичных формах медикаментозное лечение зависит от основной этиологии. Несмотря на лечение, СХ часто прогрессирует до билиарного цирроза и терминальной стадии заболевания печени, требующей трансплантации печени. Заболевание может рецидивировать после трансплантации. Для улучшения прогноза этого инвалидизирующего заболевания печени необходимо лучшее понимание патогенетических механизмов и совершенствование методов лечения.

аутоиммунный склерозирующий холангит, первичный склерозирующий холангит, дети

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