title: Anti-neutrophil cytoplasmic antibody-associated glomerulonephritis in children.
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 usefulness 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.
authors.affiliation: Victorian Paediatric Renal Service, Royal Children's Hospital, Parkville, Australia.
authors.forename: M C
authors.initials: MC
authors.lastname: Nash
authors.forename: C L
authors.initials: CL
authors.lastname: Jones
authors.forename: R G
authors.initials: RG
authors.lastname: Walker
authors.forename: H R
authors.initials: HR
authors.lastname: Powell
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies / Q000032:analysis*; D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use; D003593:Cytoplasm / Q000276:immunology*; D005260:Female; D005455:Fluorescent Antibody Technique; D005921:Glomerulonephritis / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007677:Kidney Function Tests; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D009504:Neutrophils / Q000276:immunology*; D011239:Prednisolone / Q000627:therapeutic use
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies; D003520:Cyclophosphamide; D011239:Prednisolone; D008775:Methylprednisolone