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Введение

Рецидивирующий полихондрит (РП) - это иммуноопосредованное заболевание, характеризующееся воспалением хрящевых структур и других тканей организма, особенно в области ушей, носа, глаз, суставов и дыхательных путей. Другие органы, которые могут быть вовлечены в патологический процесс, включают сосудистую систему, внутреннее ухо, центральную нервную систему и кожу. Высокий процент пациентов с генетическим заболеванием, описанным в 2020 году под названием VEXAS синдром (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) (V - это вакуолизированные миелоидные и эритроидные предшественники; E — мутации фермента Е1 в гене UBA1, которые ведут к дефициту фермента Е1 в цитоплазме; Х показывает, что ген UBA1 находится в Х хромосоме; А отражает аутовоспалительную реакцию, для которой характерно выраженное воспаление, зависимое от стероидных препаратов; S — это соматические мутации UBA1, ограниченные клетками миелоидного происхождения), имеют клинический диагноз рецидивирующий полихондрит (РП) [1].

Клинические особенности и течение рецидивирующего полихондрита значительно варьируют у разных пациентов. Малозаметные, ранние проявления нередко остаются нераспознанными в течение длительного времени. В результате диагноз часто ставится только после появления характерных признаков, таких как воспаление ушной раковины, седловидная деформация носа или другие признаки деструкции хрящевой ткани. На данный момент не существует известных клинических или лабораторных показателей, позволяющих предсказать появление специфических признаков заболевания или его общее течение.

Эпидемиология

Рецидивирующий полихондрит (РП) - редкое заболевание, которое встречается среди представителей всех рас и возрастных групп. Мужчины и женщины страдают в равной степени, однако эпидемиология этого заболевания остается недостаточно изученной из-за относительно небольшого числа пациентов. Рецидивирующий полихондрит наиболее часто встречается у лиц европеоидной расы. По данным популяционных исследований в Великобритании, Венгрии и Миннесоте, ежегодная заболеваемость оценивается в 0,71, 2,0 и 3,5 на миллион человек соответственно [2-4]. Начало заболевания чаще всего приходится на возраст от 40 до 60 лет, хотя может возникать и в детском возрасте (рис. 1) [5].

Рецидивирующий полихондрит не является наследственным заболеванием, хотя исследования указывают на вероятное наличие генетической предрасположенности к нему. В одном из отчетов о клиническом случае была высказана мысль о возможности плацентарной передачи [6]. Однако другие случаев плацентарной передачи не подтверждены, и описанная связь может быть случайной или обусловленной совпадением других обстоятельств. В целом, случаи, предполагающие плацентарную передачу, чрезвычайно редки, даже если у матери наблюдается активное мультисистемное заболевание [6].

Клинические проявления

Лабораторные исследования

Сопутствующие заболевания

Рецидивирующий полихондрит и беременность

Полезные материалы

Резюме

Список литературы