The role of the immune system in idiopathic nephrotic syndrome

被引:0
作者
Agnes Hackl
Seif El Din Abo Zed
Paul Diefenhardt
Julia Binz-Lotter
Rasmus Ehren
Lutz Thorsten Weber
机构
[1] University of Cologne,Department of Pediatrics
[2] Faculty of Medicine and University Hospital Cologne,Department of Internal Medicine II and Center for Molecular Medicine Cologne
[3] University of Cologne,undefined
[4] Faculty of Medicine and University Hospital Cologne,undefined
来源
Molecular and Cellular Pediatrics | / 8卷
关键词
Idiopathic nephrotic syndrome; T-cell dysregulation; Regulatory T-cells; Th17-cells; Podocytes; Antigen-presenting cell;
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摘要
Idiopathic nephrotic syndrome (INS) in children is characterized by massive proteinuria and hypoalbuminemia and usually responds well to steroids. However, relapses are frequent, which can require multi-drug therapy with deleterious long-term side effects. In the last decades, different hypotheses on molecular mechanisms underlying INS have been proposed and several lines of evidences strongly indicate a crucial role of the immune system in the pathogenesis of non-genetic INS. INS is traditionally considered a T-cell-mediated disorder triggered by a circulating factor, which causes the impairment of the glomerular filtration barrier and subsequent proteinuria. Additionally, the imbalance between Th17/Tregs as well as Th2/Th1 has been implicated in the pathomechanism of INS. Interestingly, B-cells have gained attention, since rituximab, an anti-CD20 antibody demonstrated a good therapeutic response in the treatment of INS. Finally, recent findings indicate that even podocytes can act as antigen-presenting cells under inflammatory stimuli and play a direct role in activating cellular pathways that cause proteinuria. Even though our knowledge on the underlying mechanisms of INS is still incomplete, it became clear that instead of a traditionally implicated cell subset or one particular molecule as a causative factor for INS, a multi-step control system including soluble factors, immune cells, and podocytes is necessary to prevent the occurrence of INS. This present review aims to provide an overview of the current knowledge on this topic, since advances in our understanding of the immunopathogenesis of INS may help drive new tailored therapeutic approaches forward.
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