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Immunopathogenesis of idiopathic nephrotic syndrome in children: two sides of the coin

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Abstract

Background

Idiopathic nephrotic syndrome is a common form of glomerular nephropathy in children, with an incidence rate of 1.15–16.9/100,000 depending on different nationalities and ethnicities. The etiological factors and mechanisms of childhood idiopathic nephrotic syndrome have not yet been fully elucidated. This review summarizes the progress of the immunopathogenesis of idiopathic nephrotic syndrome in children.

Data sources

We review the literature on the immunopathogenesis of idiopathic nephrotic syndrome in children. Databases including Medline, Scopus, and Web of Science were searched for studies published in any language with the terms “children”, “idiopathic nephrotic syndrome”, “immunopathogenesis”, “T cells”, “circulating permeability factors”, and “B cells”.

Results

Dysfunction in T lymphocytes and pathogenic circulatory factors were indicated to play key roles in the pathogenesis of idiopathic nephrotic syndrome. Recently, some studies have shown that cellular immune dysfunction may also be involved in the pathogenesis of idiopathic nephrotic syndrome.

Conclusions

Both T- and B-cell dysfunction may play significant roles in the pathogenesis of idiopathic nephrotic syndrome, like two sides of one coin, but the role of B cell seems more important than T cells.

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Funding

This study was supported by the National Natural Foundation of China (81770710), Key Research and Development Plan of Zhejiang Province (2019C03028), the Major projects jointly constructed by the Zhejiang Province, and National Health Commission (WKJ-ZJ-1908).

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Authors and Affiliations

  1. Department of Nephrology, Ningbo Women and Children’s Hospital, 339 LiutingRd, Ningbo, 315012, China

    Jing Chen & Xiao-Hui Qiao

  2. Department of Nephrology, Children’s Hospital, Zhejiang University School of Medicine, 57 Zhuganxiang, Hangzhou, 310003, China

    Jian-Hua Mao

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  1. Jing Chen
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Chen, J., Qiao, XH. & Mao, JH. Immunopathogenesis of idiopathic nephrotic syndrome in children: two sides of the coin. World J Pediatr 17, 115–122 (2021). https://doi.org/10.1007/s12519-020-00400-1

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