Treatment of steroid-resistant idiopathic nephrotic syndrome

• Published in: Archives de pédiatrie : organe officiel de la Société française de pédiatrie Vol. 16; no. 3; pp. 260 - 268
• Main Authors: Chemli, J, Harbi, A
• Format: Journal Article
• Language: French
• Published: France 01.03.2009
• Subjects: Adrenal Cortex Hormones - administration & dosage; Adrenal Cortex Hormones - adverse effects; Drug Resistance; Enzyme Inhibitors - therapeutic use; Humans; Immunosorbent Techniques; Immunosuppressive Agents - therapeutic use; Kidney Transplantation; Nephrotic Syndrome - therapy; Plasmapheresis
• ISSN: 0929-693X; 1769-664X
• DOI: 10.1016/j.arcped.2008.11.018

Idiopathic nephrotic syndrome (INS) is the most frequent glomerular nephropathy in children. The response to corticoids distinguishes steroid-sensitive nephrotic syndrome (SSINS), by far the most frequent (90% of cases), from steroid-resistant nephrotic syndrome (SRINS). The steroid resistance of nephrotic syndrome is defined by the absence of remission after a full dose of oral corticosteroid therapy for 1 month followed by 3 pulses of intravenous methylprednisolone. Actually, INS constitutes a heterogeneous nosologic entity. Currently, within the SRINS, there are 2 forms that vary greatly in their physiopathology and prognostics: immunologic or sporadic forms, which can be improved by immunosuppressive agents and the genetic or familial forms, which do not respond to any immunosuppressive therapy and usually evolve to end-stage renal insufficiency. In these genetic forms, renal transplantation is the only therapeutic alternative. The aim of this article is to review treatment of SRINS and to propose a management strategy.