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Immunopathogenesis of idiopathic nephrotic syndrome

Cellular & Molecular Immunology volume 19, pages 1429–1431 (2022)Cite this article

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Idiopathic nephrotic syndrome (INS) describes a group of rare glomerular diseases characterized by massive proteinuria and hypoalbuminemia in the absence of glomerular inflammatory lesions or immunoglobulin deposits. INS includes two main entities based on kidney biopsy findings: minimal-change nephrotic syndrome (MCNS) and focal segmental glomerulosclerosis (FSGS). The treatment of INS relies on steroids and/or immunosuppressive drugs (calcineurin inhibitors, cyclophosphamide, mycophenolate mofetil, and rituximab). Distinct clinical patterns, such as steroid sensitivity with frequent relapses (in 70–80% of cases) and primary or secondary steroid-resistant forms, can be observed. INS is considered a chronic medical condition that interferes with well-being and health-related quality of life in childhood as well as adulthood.

While genetic studies have revealed rare forms of inherited nephrotic syndrome, they failed to identify any genetic defect, except an association with some variants of class II MHC antigens, in INS with relapse [1]. Studies on genetic polymorphisms in the variable region of the T-cell receptor (TCR) β-chain have revealed selective recruitment of some β chain variable gene families in peripheral CD8+ T cells from nephrotic patients with frequent relapses [2].

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Authors and Affiliations

  1. Université Paris Est (UPEC), Faculté de Santé, UMRS 955, Créteil, F-94010, France

    B. Savas, F. Fofana, S. Le Gouvello, A. Pawlak, D. Sahali & M. Ollero

  2. AP-HP, Groupe Henri-Mondor Albert-Chenevier, Service de Néphrologie, Créteil, F-94010, France

    D. Sahali

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  1. B. Savas
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Correspondence to D. Sahali.

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Savas, B., Fofana, F., Le Gouvello, S. et al. Immunopathogenesis of idiopathic nephrotic syndrome. Cell Mol Immunol 19, 1429–1431 (2022). https://doi.org/10.1038/s41423-022-00908-8

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