Болезнь с антителами к гломерулярной базальной мембране с быстро прогрессирующим гломерулонефритом и геморрагическим альвеолитом у педиатрических пациентов (обзор литературы)

   Статья содержит обзор литературы по этиологии, патогенезу, клинике, диагностике, стратегии терапии, исходу аутоиммунного васкулита сосудов малого калибра – болезни, обусловленной антителами (АТ) к гломерулярной базальной мембране (ГБМ), манифестирующей быстро прогрессирующим гломерулонефритом (БПГН) с острым повреждением почек и геморрагическим альвеолитом у педиатрических пациентов. В обзоре представлены особенности патогенеза, клинической манифестации, лечения, исхода и прогноза болезни с АТ к ГБМ: идиопатической болезни с АТ класса IgG (IgG1 и IgG3) против неколлагенового домена-1 (NC1) α3-цепи коллагена IV типа ГБМ (аутоантигена Goodpasture); с одновременным повышением АТ к ГБМ и ANCA; болезни «de-novo» с АТ к цепям α5(IV) и α3(IV) коллагена IV типа ГБМ в почечном трансплантате при синдроме Alport у педиатрических пациентов.

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