Outcome of antineutrophil cytoplasmic autoantibodies-positive glomerulonephritis and vasculitis in children: a single-center experience

J Pediatr. 1998 Feb;132(2):325-8. doi: 10.1016/s0022-3476(98)70453-6.


Vasculitis associated with antineutrophil cytoplasmic autoantibodies (ANCA) can be accompanied by a focal and necrotizing glomerulonephritis that carries a high morbidity. As many as 60% of reported children with ANCA-associated glomerulonephritis progress to end-stage renal disease. Seven children (13.0+/-0.89 years, mean age +/- SEM) with both a focal and necrotizing glomerulonephritis and a positive ANCA titer are described. Presenting symptoms were constitutional (100%) and sinopulmonary (71%); additional renal features included microscopic hematuria (100%), proteinuria (71%), and renal insufficiency (71%). Acute therapy (0 to 2 weeks from diagnosis) included intravenous corticosteroids and intravenous cyclophosphamide for all patients. Induction therapy (2 weeks to 6 months from diagnosis) consisted of cyclophosphamide (100%) and daily corticosteroids (86%) for a minimum of 6 months. Maintenance therapy that followed 6 months of induction therapy consisted of alternate day steroids (100%) combined with either oral azathioprine (50%) or oral cyclophosphamide (50%). Long-term follow-up for 48+/-12 months in all seven patients revealed that only one (14%) patient had end-stage renal disease, whereas the remaining patients had microscopic hematuria (100%), proteinuria (50%), and renal insufficiency (33%). These findings suggest that early recognition and aggressive treatment of children with ANCA-associated glomerulonephritis and vasculitis may result in an improved renal outcome compared with previous reports.

MeSH terms

  • Adolescent
  • Antibodies, Antineutrophil Cytoplasmic
  • Child
  • Cyclophosphamide / therapeutic use
  • Female
  • Glomerulonephritis / complications
  • Glomerulonephritis / immunology
  • Glomerulonephritis / physiopathology
  • Glomerulonephritis / therapy*
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Kidney Function Tests
  • Male
  • Methylprednisolone / therapeutic use
  • Retrospective Studies
  • Vasculitis / complications
  • Vasculitis / immunology
  • Vasculitis / physiopathology
  • Vasculitis / therapy*


  • Antibodies, Antineutrophil Cytoplasmic
  • Glucocorticoids
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Methylprednisolone