New insights into the pathophysiology of idiopathic nephrotic syndrome.
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.
Bruneau
Sarah
S
INSERM, U643, Nantes, F44093, France.
Dantal
Jacques
J
D000293:Adolescent; D000328:Adult; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D002648:Child; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D007676:Kidney Failure, Chronic / Q000601:surgery; D016030:Kidney Transplantation; D008297:Male; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000188:drug therapy* / Q000276:immunology*; D011183:Postoperative Complications / Q000188:drug therapy* / Q000276:immunology*; D012008:Recurrence; D000069283:Rituximab; D016176:T-Lymphocyte Subsets / Q000276:immunology*; D016896:Treatment Outcome
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab