Abstract
This report describes an 8-year-old girl with crescentic IgA nephropathy. Her father has also suffered from renal insufficiency due to IgA nephropathy and been treated with hemodialysis. Marked proteinuria and hematuria was pointed out by a follow-up screening. She was referred to our hospital for further evaluation of proteinuria and hematuria with red blood casts. First renal biopsy showed crescentic IgA nephropathy with mesangial cell proliferation and mesangial deposition of IgA. Intensive treatment including methylprednisolone pulse therapy and combination with oral prednisolone and cyclophosphamide ameliorated active pathological findings such as cellular crescents and mesangial cell proliferation as indicated by 2nd biopsy. Deterioration of renal function was not observed during the course of treatment. Proteinuria was reduced and disappeared by intensive treatment and combination therapy of angiotensin-converting enzyme inhibitor and angiotensin receptor blocker for 7 months. In conclusion, early start of intensive treatment is very important for the reverse of active pathological lesion of crescentic IgA nephropathy.