nefrНефрологияNephrology (Saint-Petersburg)1561-62742541-9439Pavlov First Saint-Petersburg State Medical University10.36485/1561-6274-2022-26-3-9-18nefr-2134Research ArticleОБЗОРЫ И ЛЕКЦИИREVIEWS AND LECTURESБолезнь с антителами к гломерулярной базальной мембране с быстро прогрессирующим гломерулонефритом и геморрагическим альвеолитом у педиатрических пациентов (обзор литературы)Anti–glomerular basement membrane disease with rapidly progressive glomerulonephritis and hemorrhagic alveolitis in pediatric patients (reviev of literature)https://orcid.org/0000-0002-9415-4785СавенковаН. Д.SavenkovaN. D.

Надежда Дмитриевна Савенкова, проф., д-р мед. наук, заведующая кафедрой

кафедра факультетской педиатрии

194100

ул. Литовская, д. 2

Санкт–Петербург

тел.: (812) 4165286

Nadezhda D. Savenkova, Professor, Doctor of Medical Sciences, Head of the Department

Department of Faculty Pediatrics

194100

Litovskaya str., 2

Saint-Petersburg

phone: (812) 4165286

savenkova.n.spb@mail.ruhttps://orcid.org/0000-0001-8692-0315ПапаянК. А.PapayanK. A.

Карина Альбертовна Папаян, доц., канд. мед. наук

кафедра факультетской педиатрии

194100

ул. Литовская, д. 2

Санкт–Петербург

тел.: (812) 4165286

Karina A. Papayan, Associate Professor, Candidate of Medical Sciences

Department of Faculty Pediatrics

194100

Litovskaya str., 2

Saint-Petersburg

phone: (812) 4165286

papaya@inbox.ruСанкт-Петербургский государственный педиатрический медицинский университетРоссияSt. Petersburg State Pediatric Medical UniversityRussian Federation202209092022263918Copyright © Савенкова Н.Д., Папаян К.А., 20222022Савенкова Н.Д., Папаян К.А.Savenkova N.D., Papayan K.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephrolog.ru/jour/article/view/2134

   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.

   The article is devoted to controversial issues of autoimmune small vessel vasculitis with kidney and lung damage. Modern data on the features of pathogenesis, clinical manifestation, treatment, outcome, and prognosis of the disease with AT to GBM have presented: an idiopathic disease with AT of class IgG (IgG1 and IgG3) against non-collagen domain-1 (NC1) α3-chain ofcollagen type IV GBM (Goodpasture autoantigen); with the simultaneous increase in AT to GBM and ANCA; "de novo" diseases with AT to the α5(IV) and α3(IV) chains of GBM type IV collagen in renal graft with Alport syndrome in pediatric patients.

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The authors declare that there are no conflicts of interest present.