nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное общество10.28996/2618-9801-2021-2-160-173nid-26Research ArticleОБЗОРЫ И ЛЕКЦИИREVIEWS AND LECTURESСовременные представления о первичном фокальном сегментарном гломерулосклерозе у взрослых: патогенез и подходы к лечению. Обзор литературыModern view on primary focal segmental glomerulosclerosis in adults: pathogenesis and approaches to therapy. ReviewЧеботареваН. В.ChebotarevaN. V.natasha_tcheb@mail.ruЛысенкоЛ. В.LysenkoL. V.noemail@neicon.ruФедеральное государственное автономное образовательное учреждение высшего образования Первый Московский государственный медицинский университет имени И.М. СеченоваРоссияI.M. Sechenov First Moscow State Medical University (Sechenov University)Russian Federation202121062024232160173Copyright © Чеботарева Н.В., Лысенко Л.В., 20242024Чеботарева Н.В., Лысенко Л.В.Chebotareva N.V., Lysenko L.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephro.ru/jour/article/view/26

Введение: первичный фокальный сегментарный гломерулосклероз является морфологическим вариантом нефропатии, в основе которой лежит повреждение подоцитов. Патогенез первичного ФСГС изучается с 90-х годов, однако полностью не расшифрован до настоящего времени. Вместе с тем, накоплено большое количество данных, позволяющих сформулировать определенную концепцию развития заболевания. Цель настоящего обзора представить современный взгляд на патогенез и подходы к терапии первичного ФСГС у взрослых. Основные сведения: в обзоре представлен современный взгляд на патогенез первичного фокального сегментарного гломерулосклероза. Обсуждается роль подоцитов и париетальных эпителиальных клеток в формировании очагов склероза и прогрессировании заболевания. Особое внимание уделено предполагаемым молекулам-кандидатам на роль циркулирующих факторов проницаемости: растворимого рецептора активатора плазминогена (suPAR), кардиотрофин-подобного фактора-1 (CLCF-1), CD80 (B7-1) и механизмам их действия. Повышенный уровень этих факторов выявляют в сыворотке крови и на поверхности подоцитов у больных ФСГС. Согласно современной концепции, патогенез первичного ФСГС связан с нарушением адаптивного иммунного ответа. Представлены данные о том, что дисбаланс определенных Т-клеточных подклассов участвует в патогенезе заболевания. Повышение Th17 лимфоцитов и снижение количества Т-регуляторных клеток может оказаться решающим для развития первичного ФСГС. Имеет также значение активация В-клеток, о чем свидетельствуют непосредственное влияние В-лимфоцитов на развитие протеинурии, выявление циркулирующих антител и эффективность применения ритуксимаба у больных с ФСГС. Обсуждается возможная роль анти-CD40-антител и антител к убиквитин карбоксильной гидролазе L1 в развитии первичного ФСГС. Прямо или через продукцию факторов проницаемости эти механизмы ведут к повреждению подоцитов. В отдельном разделе представлен алгоритм иммуносупрессивной терапии: глюкокортикостероидов и цитостатиков. Рассмотрены перспективы терапии ФСГС с учетом предполагаемых патогенетических механизмов заболевания.

Introduction: primary focal segmental glomerulosclerosis is a morphological variant of chronic nephropathy based on podocyte damage. The pathogenesis of primary FSGS has been studied since the 90s but has not been fully understood until now. At the same time, a large amount of data has been accumulated allowing one to build a certain concept of the disease. The review aimed to present a modern view on the pathogenesis and approaches to the treatment of primary FSGS in adults. Main information: the review presents current view on the pathogenesis of primary focal segmental glomerulosclerosis. The role of podocytes and parietal epithelial cells in the formation of sclerosis foci and disease progression is discussed. The possible candidate molecules of circulating permeability factors and mechanisms of their action are reviewed: soluble plasminogen activator receptor (suPAR), cardiotrophin-like factor-1 (CLCF-1), CD80 (B7-1). An increased level of these factors was detected in the serum and on the surface of podocytes in FSGS patients. According to the modern concept, the pathogenesis of primary FSGS is associated with the adaptive immune response. The imbalance of certain T-cell subclasses plays a role in the pathogenesis of the disease. In particular, an increase in Th17 lymphocytes and a decrease in the number of T regulatory cells are believed to be crucial for the development of primary FSGS. The activation of B cells is also important, as evidenced by the direct effect of B lymphocytes on proteinuria, the detection of circulating antibodies, and the success of rituximab in FSGS patients. The possible role of anti-CD40 antibodies and ubiquitin carboxyl hydrolase L1 antibodies in primary FSGS is discussed. These mechanisms lead to damage to podocytes directly or through the production of permeability factors. A separate section describes the algorithm of immunosuppressive therapy: glucocorticosteroids and cytostatics, as well as future treatment approaches in FSGS patients.

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