New insights into the pathophysiology of idiopathic nephrotic syndrome

Clin Immunol. 2009 Oct;133(1):13-21. doi: 10.1016/j.clim.2009.03.532. Epub 2009 May 1.


Corticoresistant idiopathic nephrotic syndrome (INS) is a glomerulopathy of unknown etiology whose original aspect is its recurrence after kidney transplantation in 30 to 50% of patients with end-stage renal disease. This suggests the involvement of circulating factors that would alter the glomerular filtration barrier, but whose nature remains elusive. Although a T cell immune origin has been suggested, the actual role of these cells in INS recurrence is still unclear. Here we present an 8-year-old patient with corticoresistant INS who developed a recurrence of her initial disease after kidney transplantation. Rituximab therapy was proposed 11 months after transplantation; although no immediate effect was induced, a slow but persistent decrease in proteinuria began a few months after Rituximab infusions despite cessation of plasma exchanges and steroid therapy. The pathophysiology of INS and the putative mechanisms of action of Rituximab are discussed.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Antibodies, Monoclonal / therapeutic use*
  • Antibodies, Monoclonal, Murine-Derived
  • Child
  • Female
  • Humans
  • Immunologic Factors / therapeutic use*
  • Kidney Failure, Chronic / surgery
  • Kidney Transplantation
  • Male
  • Middle Aged
  • Nephrotic Syndrome / drug therapy*
  • Nephrotic Syndrome / immunology*
  • Postoperative Complications / drug therapy*
  • Postoperative Complications / immunology*
  • Recurrence
  • Rituximab
  • T-Lymphocyte Subsets / immunology*
  • Treatment Outcome


  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Murine-Derived
  • Immunologic Factors
  • Rituximab