Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2018 Apr;33(4):573-584.
doi: 10.1007/s00467-017-3677-5. Epub 2017 Apr 27.

Immunology of idiopathic nephrotic syndrome

Manuela Colucci  1 , Giorgia Corpetti  1 , Francesco Emma  1 , Marina Vivarelli  2
Affiliations
Review

Immunology of idiopathic nephrotic syndrome

Manuela Colucci et al. Pediatr Nephrol. 2018 Apr.

Abstract

The pathogenesis of idiopathic nephrotic syndrome (INS) is as yet unknown, but several lines of evidence indicate that the immune system may play a crucial pathogenic role in non-genetic INS. The most important of these are, first, the effectiveness of therapy based on immunosuppression and, second, a vast body of data derived both from experimental models and from patient studies that implicate T cells and more recently B cells as major players in INS pathogenesis. However, recent findings also suggest a direct role of podocytes as drivers of the disease process, and the interplay between the glomerulus and the immune system is still being elucidated. In this review we provide an overview of current knowledge on the role of different components of the immune system in determining disease. Advances in our understanding of the pathogenesis of INS may help drive new, more tailored therapeutic approaches.

Keywords: B cells; Circulating permeability factors; Idiopathic nephrotic syndrome; Immunosuppressive therapy; Podocytes; Rituximab; T cells.

PubMed Disclaimer

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. J Am Soc Nephrol. 2004 Sep;15(9):2246-8 - PubMed
    1. Clin Exp Immunol. 2010 Feb;159(2):199-207 - PubMed
    1. Semin Immunopathol. 2014 Jul;36(4):421-9 - PubMed
    1. J Am Soc Nephrol. 2016 Jul;27(7):1890-900 - PubMed
    1. Kidney Int. 2005 Dec;68(6):2473-83 - PubMed

MeSH terms

Substances

Supplementary concepts

LinkOut - more resources