title: Outcome of antineutrophil cytoplasmic autoantibodies-positive glomerulonephritis and vasculitis in children: a single-center experience.
abstract: 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.
authors.affiliation: Department of Pediatrics, University of Michigan, Ann Arbor, USA.
authors.forename: R P
authors.initials: RP
authors.lastname: Valentini
authors.forename: W E
authors.initials: WE
authors.lastname: Smoyer
authors.forename: A B
authors.initials: AB
authors.lastname: Sedman
authors.forename: D B
authors.initials: DB
authors.lastname: Kershaw
authors.forename: M J
authors.initials: MJ
authors.lastname: Gregory
authors.forename: T E
authors.initials: TE
authors.lastname: Bunchman
mesh_terms: D000293:Adolescent; D019268:Antibodies, Antineutrophil Cytoplasmic; D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005921:Glomerulonephritis / Q000150:complications / Q000276:immunology / Q000503:physiopathology / Q000628:therapy*; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007677:Kidney Function Tests; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D012189:Retrospective Studies; D014657:Vasculitis / Q000150:complications / Q000276:immunology / Q000503:physiopathology / Q000628:therapy*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008775:Methylprednisolone