Outcome of Henoch-Schönlein purpura nephritis treated with long-term immunosuppression

Pediatr Nephrol. 2007 Oct;22(10):1717-22. doi: 10.1007/s00467-007-0557-4. Epub 2007 Jul 24.

Abstract

This retrospective study investigated the outcome of 27 children (19 male) with Henoch-Schönlein purpura nephritis (HSN) of International Study of Kidney Disease in Children (ISKDC) grade 3b or higher treated with long-term immunosuppressive therapy in a single centre over a 10-year period. The mean age at presentation was 9.7 years. The median estimated glomerular filtration rate (eGFR) was 91.3 ml/min per 1.73 m(2), with the median urine protein creatinine ratio (UP:UC) 556 mg/mmol. The treatment protocol comprised daily steroids and cyclophosphamide for 8-12 weeks followed by azathioprine and a reducing regimen of alternate-day steroids for 8-12 months. After a mean follow-up period of 7 years following presentation, 37% made a complete recovery, 40.7% had persistent proteinuria, 7.4% had persistent proteinuria and were on antihypertensive therapy and 14.8% had progressed to end-stage kidney failure (ESKF). Children with poor outcome were older at presentation (p 0.005), had more crescents (p 0.015) and had heavier proteinuria 6 months post initial biopsy (p 0.023). All of the four children with ESKF had nephrotic range proteinuria and greater than 50% crescents on initial biopsy. Despite long-term immunosuppression, the majority of children with HSN grade 3b or higher will have persistent renal abnormalities on long-term follow-up.

MeSH terms

  • Adolescent
  • Adrenal Cortex Hormones / therapeutic use
  • Age of Onset
  • Azathioprine / therapeutic use*
  • Child
  • Cyclophosphamide / therapeutic use*
  • Female
  • Follow-Up Studies
  • Glomerular Filtration Rate
  • Humans
  • IgA Vasculitis / complications
  • IgA Vasculitis / drug therapy*
  • Immunosuppressive Agents / therapeutic use*
  • Immunotherapy*
  • Male
  • Nephritis / complications
  • Nephritis / immunology*
  • Proteinuria
  • Retrospective Studies
  • Treatment Outcome

Substances

  • Adrenal Cortex Hormones
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Azathioprine