A Modified Regimen of Methylprednisolone, Cyclophosphamide, and Mycophenolic Acid for Severe IgA Vasculitis with Nephritis in a Child

Saudi J Kidney Dis Transpl. 2020 Sep-Oct;31(5):1117-1124. doi: 10.4103/1319-2442.301180.

Abstract

IgA vasculitis with nephritis (IgAVN), which was formerly known as Henoch-Schonlein purpura nephritis, commonly manifests with mild symptoms. However, in rare circumstances, IgAVN in children can progress to kidney failure. Despite the successful treatment of severe IgAVN with a combination of immunosuppressive medications including corticosteroids, no consensus has been established for IgAVN treatment. Here, we present a case of severe IgAVN in an eight-year-old Indonesian boy who was treated with simultaneous methylprednisolone, cyclophosphamide, and mycophenolic acid. He experienced recovery of kidney function within one month, while proteinuria resolved in five months, and hematuria resolved within a year after treatment initiation. No recurrences were noted during the two-year follow-up. Although our immunosuppressive regimen may seem very potent, it was shown to have tolerable side effects and could be beneficial for kidney recovery. Importantly, they have also been shown to prevent progression to chronic kidney disease in children with severe IgAVN.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Cyclophosphamide* / administration & dosage
  • Cyclophosphamide* / therapeutic use
  • Glomerulonephritis, IGA / diagnosis
  • Glomerulonephritis, IGA / drug therapy*
  • Glomerulonephritis, IGA / pathology
  • Humans
  • Immunosuppressive Agents / administration & dosage
  • Immunosuppressive Agents / therapeutic use
  • Kidney Glomerulus / pathology
  • Male
  • Methylprednisolone* / administration & dosage
  • Methylprednisolone* / therapeutic use
  • Mycophenolic Acid* / administration & dosage
  • Mycophenolic Acid* / therapeutic use
  • Vasculitis / diagnosis
  • Vasculitis / drug therapy*
  • Vasculitis / pathology

Substances

  • Immunosuppressive Agents
  • Cyclophosphamide
  • Mycophenolic Acid
  • Methylprednisolone