title: Antineutrophil cytoplasmic autoantibody-associated glomerulonephritis in children.
abstract: Aretrospective investigation was conducted by members of the Japanese Society for Pediatric Nephrology from 1990 to 1997 to define the clinical features and outcome of antineutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis in children. Thirty-four ANCA-seropositive Japanese pediatric patients with biopsy-proven pauci-immune necrotizing crescentic glomerulonephritis were identified. Of these, 3 cases associated with Wegener's granulomatosis were excluded because of the small sample size. Among the 31 patients studied, 10 had a diagnosis of necrotizing crescentic glomerulonephritis alone and 21 had microscopic polyangiitis. Females predominated (87%), and the median age at onset was 12 yr. Twenty-six patients received treatment with cyclophosphamide and corticosteroids, and five patients received treatment with corticosteroids alone; 84% of patients achieved remission, and 39% of responders relapsed in a median of 24 mo. ANCA titers correlated with response to treatment and disease activity, with some exceptions. Patients were followed for a median of 42 mo (range, 3 to 96 mo). Nine of 31 patients (29.0%) progressed to end-stage renal disease, 6 (19.4%) had reduced renal function, and 15 (48.4%) had normal renal function at the last observation. One patient (3.2%) died from cytomegalovirus infection 3 mo after initiation of therapy. Life-table analysis showed 75% renal survival at 39 mo. Patients who subsequently developed end-stage renal disease (n = 9) had significantly higher average peak serum creatinine levels and more chronic pathologic lesions at diagnosis compared with patients with favorable renal outcome (n = 15). In conclusion, our clinical experience suggests that the clinical disease spectrum of ANCA-associated glomerulonephritis is similar in pediatric and adult patients, but there is a female predominance in children.
authors: Department of Pediatric Nephrology, Tokyo Women's Medical University, School of Medicine, Tokyo, Japan.
authors: Motoshi
authors: M
authors: Hattori
authors: Department of Pediatrics, National Chiba Higashi Hospital, Chiba, Japan.
authors: Hideaki
authors: H
authors: Kurayama
authors: Department of Pediatrics, St. Marianna University, School of Medicine, Kawasaki, Japan.
authors: Yasushi
authors: Y
authors: Koitabashi
mesh_terms: D000293:Adolescent; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005921:Glomerulonephritis / Q000150:complications / Q000276:immunology* / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D007668:Kidney / Q000503:physiopathology; D015993:Life Tables; D008297:Male; D011379:Prognosis; D012008:Recurrence
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic