Antineutrophil cytoplasmic autoantibody-associated glomerulonephritis in children

A retrospective investigation was conducted by members of the Japanese Soci ety for Pediatric Nephrology from 1990 to 1997 to define the clinical featu res and outcome of antineutrophil cytoplasmic autoantibody (ANCA)-associate d glomerulonephritis in children. Thirty-four ANCA-seropositive Japanese pe diatric patients with biopsy-proven pauci-immune necrotizing crescentic glo merulonephritis 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 glomerulo nephritis alone and 21 had microscopic polyangiitis. Females predominated ( 87%), and the median age at onset was 12 yr. Twenty-six patients received t reatment with cyclophosphamide and corticosteroids, and five patients recei ved treatment with corticosteroids alone; 84% of patients achieved remissio n, and 39% of responders relapsed in a median of 24 mo. ANCA titers correla ted 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 3 1 patients (29.0%) progressed to end-stage renal disease, 6 (19.4%) had red uced renal function, and 15 (48.4%) had normal renal function at the last o bservation. One patient (3.2%) died from cytomegalovirus infection 3 mo aft er 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 chro nic pathologic lesions at diagnosis compared with patients with favorable r enal outcome (n = 15). In conclusion, our clinical experience suggests that the clinical disease spectrum of ANCA-associated glomerulonephritis is sim ilar in pediatric and adult patients, but there is a female predominance in children.