Pediatric Nephrology

, Volume 7, Issue 1, pp 11–14

Anti-neutrophil cytoplasmic antibody-associated glomerulonephritis in children

  • Margot C. Nash
  • Colin L. Jones
  • Rowan G. Walker
  • Harley R. Powell
Original Article

DOI: 10.1007/BF00861551

Cite this article as:
Nash, M.C., Jones, C.L., Walker, R.G. et al. Pediatr Nephrol (1993) 7: 11. doi:10.1007/BF00861551
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Abstract

Two cases of anti-neutrophil cytoplasmic antibody (ANCA)-associated necrotizing and crescentic glomerulonephritis are reported. A 12-year-old girl and a 10-year-old boy presented with polyarthritis, anaemia, haematuria, proteinuria, impaired renal function, anorexia, nausea, marked loss of weight and lethargy. The boy also had a vasculitic rash and anterior uveitis. Both children had diffuse cytoplasmic ANCA identified by indirect immunofluorescence and confirmed by specific enzyme-linked immunosorbent assay. Renal biopsies showed severe focal and segmental necrotizing glomerulonephritis with 100% crescents. They were treated with plasma exchange, prednisolone, cyclophosphamide and heparin. Within 1 month of commencing treatment, both had normal serum creatinine concentrations and ANCA was not detectable. Renal biopsies 6 weeks following commencement of treatment revealed quiescent disease, although up to 40% of glomeruli were sclerosed or had fibrous crescents. Following cessation of cyclophosphamide and heparin after 7 months and reduction in steroid dose, a biopsy at 10 months in the boy revealed quiescent disease, but the girl had recurrent disease associated with reappearance of a low titre of ANCA and small cellular crescents in 20% of the glomeruli. These cases reflect the potential usefulnes of ANCA determination for categorizing paediatric patients, helping in the selection of therapy and as a possible marker of disease activity, similar to the experience in adults.

Key words

Crescentic glomerulonephritis Anti-neutrophil cytoplasmic antibody Microscopic polyarteritis 

Copyright information

© IPNA 1993

Authors and Affiliations

  • Margot C. Nash
    • 1
  • Colin L. Jones
    • 1
  • Rowan G. Walker
    • 1
  • Harley R. Powell
    • 1
  1. 1.Victorian Paediatric Renal ServiceRoyal Children's HospitalParkvilleAustralia
  2. 2.Department of NephrologyRoyal Children's HospitalParkvilleAustralia

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