nefrНефрологияNephrology (Saint-Petersburg)1561-62742541-9439Pavlov First Saint-Petersburg State Medical Universitynefr-15Research ArticleОБЗОРЫ И ЛЕКЦИИREVIEWS AND LECTURESСовременные представления о патогенезе иммуноглобулин А-нефропатииModern insights on IgA nephropathy pathogenesisКомиссаровК. С.KomissarovK. S.kirill_ka@tut.byЮркевичМ. Ю.YurkevichM. Ju.noemail@neicon.ruЗафранскаяМ. М.ZafranskayaM. M.noemail@neicon.ruПилотовичВ. С.PilotovichV. S.noemail@neicon.ruГосударственное учреждение образования «Белорусская медицинская академия последипломного образования»Russian Federation2014010320141824754Copyright © Комиссаров К.С., Юркевич М.Ю., Зафранская М.М., Пилотович В.С., 20142014Комиссаров К.С., Юркевич М.Ю., Зафранская М.М., Пилотович В.С.Komissarov K.S., Yurkevich M.J., Zafranskaya M.M., Pilotovich V.S.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/15

IgA-нефропатия (IgAH является самой частой формой гломерулонефрита во всем мире. До настоящего времени этиология и патогенез IgAН остаются до конца не изученными. В качестве инициирующего фактора рассматривается продукция «патогенного» IgA с последующим формированием иммунных комплексов, имеющих повышенное сродство к мезангиальным клеткам (МК) почечных клубочков. У 15-20% пациентов мезангиальные депозиты содержат секреторный IgA1, кроме того для них характерна активация системы комплемента по лектиновому пути. У большинства пациентов с gAН (70-80%) в мезангиуме выявляется депонирование сывороточного IgA1, имеющего дефект галактозилирования. Агалактозилированные О-гликаны выступают как аутоантигены, в ответ на которые продуцируются анти-гликановые антитела класса иммуноглобулинов G. Образовавшиеся крупные IgA-содержащие макромолекулы не эффективно выводятся из циркуляции за счет ретикуло-эндотелиальной системы, в результате чего фильтруются и депонируются в мезангиуме почечных клубочков. Следующим этапом в развитие IgAН является взаимодействие IgA1-депозитов с клетками мезангиума, в результате чего происходит их пролиферация и индуцируется гиперпродукция мезангиального матрикса.

IgA-nephropathy (IgAN) is one of the most common forms of the glomerulonephritis in the world. The aetiology and pathogenesis of IgAN are still unknown. The production of «pathogenic» IgA followed by the formation of immune complexes with high affinity to mesangial cells (MC) of the glomeruli is considered as an initiating factor. In 15-20% of patients, mesangial depositions content secretory IgA1 and this pattern is associated with activation of complement by the lectin path. In the majority of patients with IgAN (70-80%) IgA1 depositions having galactose defect are detected in mesanium. O-glycans without galactose act as autoantigenes these stimulate the producing of antiglacans antibodies from immunoglobulin G class. Such macromolecules containing IgA immunocomplex are not effectively excreted from circulation by reticuloendothelial system that is why they are filtrated and are accumulated in glomerular mesangium. The next stage of IgAN is the interaction between IgA1 deposits and mesangium that stimulates cells proliferation and increases mesangial matrix synthesis.

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