Treatment of steroid-resistant nephrotic syndrome in the genomic era.

Journal Article (Journal Article;Review)

The pathogenesis of steroid-resistant nephrotic syndrome (SRNS) is not completely known. Recent advances in genomics have elucidated some of the molecular mechanisms and pathophysiology of the disease. More than 50 monogenic causes of SRNS have been identified; however, these genes are responsible for only a small fraction of SRNS in outbred populations. There are currently no guidelines for genetic testing in SRNS, but evidence from the literature suggests that testing should be guided by the genetic architecture of the disease in the population. Notably, most genetic forms of SRNS do not respond to current immunosuppressive therapies; however, a small subset of patients with monogenic SRNS will achieve partial or complete remission with specific immunomodulatory agents, presumably due to non-immunosuppressive effects of these agents. We suggest a pragmatic approach to the therapy of genetic SRNS, as there is no evidence-based algorithm for the management of the disease.

Full Text

Duke Authors

Cited Authors

  • Bensimhon, AR; Williams, AE; Gbadegesin, RA

Published Date

  • November 2019

Published In

Volume / Issue

  • 34 / 11

Start / End Page

  • 2279 - 2293

PubMed ID

  • 30280213

Pubmed Central ID

  • PMC6445770

Electronic International Standard Serial Number (EISSN)

  • 1432-198X

Digital Object Identifier (DOI)

  • 10.1007/s00467-018-4093-1


  • eng

Conference Location

  • Germany