A 12-year-old girl with hematuria and proteinuria in school urinary screening was referred to us. Physical examination revealed no extrarenal lesions. Laboratory findings revealed impaired renal function and the elevation of serum MPO-ANCA (anti-neutrophil cytoplasmic antibody) level. Kidney biopsy showed necrotizing crescentic glomerulonephritis. From these findings, ANCA associated nephritis was made. Early remission was achieved with the combination treatment of methylprednisolone pulse therapy and rituximab. Long term remission without severe complications has been achieved with the maintenance treatment with the combination of prednisolone and azathioprine. Rituximab might be useful to achieve early remission in ANCA associated nephritis.
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