Remission of steroid- and CyA-resistant nephrotic syndrome using multiple drug immunosuppression

Pediatr Nephrol. 2007 Oct;22(10):1723-6. doi: 10.1007/s00467-007-0551-x. Epub 2007 Jul 17.

Abstract

Nephrotic proteinuria in minimal change disease (MCD) is supposed to be due to a circulating factor of immunologic origin. End-stage renal failure occurs if both steroids and immunosuppressive drugs remain ineffective. Three children (2 years, 3 years, and 6 years of age) with secondary steroid-resistant nephrotic syndrome (NS) were included, as they remained resistant to 30 days of treatment with prednisone (60 mg/m(2) per day), three pulses of methylprednisolone (1 g/1.73 m(2)) followed by oral administration of CyA 7.5 mg/kg per day over 2 months, and 1 month of intravenous (i.v.) administration of cyclosporine (blood level 500-600 ng/ml). All three patients were partially responsive to methylprednisolone pulses, with an increase of serum albumin by 100%. They were treated with plasma exchanges, cyclophosphamide and cyclosporine A, both given orally, pefloxacin and methylprednisolone pulses followed by orally administered prednisone. All three patients went into remission within 2 to 5 weeks. The character of their NS changed to a steroid-sensitive one. There were no significant side effects from the therapy. They had normal renal function, normal blood pressure and no residual proteinuria. A combination of plasmapheresis and multiple immunosuppressive medications was effective in producing remission of minimal change NS in three children who were previously resistant to glucocorticoids and cyclosporine.

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use*
  • Child
  • Child, Preschool
  • Cyclosporine / therapeutic use*
  • Drug Resistance
  • Drug Therapy, Combination
  • Female
  • Glomerular Filtration Rate
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Male
  • Nephrotic Syndrome / drug therapy*
  • Nephrotic Syndrome / immunology
  • Proteinuria / etiology
  • Proteinuria / immunology
  • Retrospective Studies
  • Treatment Outcome

Substances

  • Adrenal Cortex Hormones
  • Immunosuppressive Agents
  • Cyclosporine