Клинический случай IgG4-ассоциированного заболевания

IgG4-связанное заболевание - крайне редкое, характеризующееся воспалительной инфильтрацией различных органов с преобладанием IgG4-положительных плазмоцитов, фиброзом и повышением концентрации IgG4 в крови. Подходы к его терапии не оптимизированы, особенно в педиатрической группе пациентов. В статье обсуждается случай IgG4-связанного заболевания орбиты у мальчика 13 лет, рассмотрен новый вариант терапевтического подхода при этой патологии с применением ингибитора JAK-киназ - руксолитиниба. Представлен анализ современного состояния проблемы IgG4-связанного заболевания в мире, затрагивающий аспекты терминологии, исторические справки, патогенез, проблемы диагностики и клинические проявления.

IgG4-связанное заболевание, дети, орбита, лечение, ингибитор JAK-киназ

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