Is cyclophosphamide effective in patients with IgM-positive minimal change disease?

Pediatr Nephrol. 2012 Dec;27(12):2227-31. doi: 10.1007/s00467-012-2234-5. Epub 2012 Jun 24.

Abstract

Background: We analyzed the impact of immunoglobulin M (IgM) positivity on the relapse-free interval post completed course of cyclophosphamide (CYC) treatment in patients with steroid-dependent nephrotic syndrome (SDNS) and minimal change disease (MCD).

Methods: This was a retrospective chart review of all children who received CYC for SDNS and MCD between 1988 and 2009. Patients were divided into three groups based on kidney biopsy: MCD without immunoglobulin M (IgM) positivity (IgM-), MCD with IgM-positive immunofluorescence (IF) only (IgM+), and MCD with IgM-positive IF and electron-dense deposits on electron microscopy (IgM++). The relapse-free time interval to the first relapse post-CYC therapy or up to 48 months of follow-up (if no relapse occurred) was used for survival analysis.

Results: Forty children aged 1.5-12.3 years (15 were IgM-, 16 were IgM+, 9 were IgM++) received a cumulative CYC dose of 175 ± 30 mg/kg. The overall relapse-free survival time was 75 % at 12 months, 64 % at 24 months, 59 % at 36 months, and 56 % at 48 months, with no significant differences between the IgM groups (p = 0.80).

Conclusions: Based on our results, we conclude that more than 50% of our SDNS patients with MCD remained relapse-free 4 years post-CYC treatment. No significant difference in the response to CYC was observed between patients with or without IgM positivity.

MeSH terms

  • Child
  • Child, Preschool
  • Cyclophosphamide / therapeutic use*
  • Disease-Free Survival
  • Female
  • Humans
  • Immunoglobulin M / immunology
  • Immunosuppressive Agents / therapeutic use*
  • Infant
  • Male
  • Nephrosis, Lipoid / drug therapy*
  • Nephrosis, Lipoid / immunology
  • Nephrosis, Lipoid / mortality
  • Nephrotic Syndrome / drug therapy*
  • Nephrotic Syndrome / immunology
  • Nephrotic Syndrome / mortality
  • Retrospective Studies
  • Treatment Outcome

Substances

  • Immunoglobulin M
  • Immunosuppressive Agents
  • Cyclophosphamide