An 11-year-old girl with antineutrophil cytoplasmic antibody-associated glomerulonephritis identified by a school urinary screening program

Abstract

Antineutrophil cytoplasmic autoantibody-associated glomerulonephritis (GN) in childhood is rare and has a poor prognosis. We report an 11-year-old girl with renallimited antineutrophil cytoplasmic autoantibody-associated vasculitis. Proteinuria and hematuria were first detected by a school urinary screening (SUS) program. Histopathological examination revealed pauci-immune necrotizing GN. She did not display purpura or peripheral neuropathy. She was diagnosed with antineutrophil cytoplasmic autoantibodyassociated GN based on proteinuria, high serum titers of antineutrophil cytoplasmic autoantibodies (ANCAs), and pauci-immune necrotizing GN. The patient was treated with combination therapy, consisting of methylprednisolone and urokinase pulse, prednisolone, mizoribine (MZB), warfarin, and dilazep hydrochloride. At 2 months after treatment, urinary protein excretion was decreased and the hematuria had disappeared, while the serum titer of ANCAs was also decreased. The dose of prednisolone was tapered, and proteinuria and hematuria later disappeared at 9 months after treatment. In conclusion, we reported an 11-year-old girl with renal-limited antineutrophil cytoplasmic autoantibodyassociated vasculitis early identified by a SUS program and treated with multi-drug combination therapy including MZB. On the basis of our results, we believe that a SUS programs may be effective for the early identification and treatment of children with renal-limited antineutrophil cytoplasmic autoantibody-associated vasculitis.

Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement

“…Nonetheless, we are still not very optimistic for the long-term consequences of renal involvement. There are patients reported to have early diagnosis and treatment by school urine screening programs in some countries 2. In the literature, there are very few patients with isolated renal involvement and ANCA positivity.…”

“…In a limited number of cases, ANCA is negative and the renal involvement is isolated. In some cases, drug induced crescentic GN secondary to penicillamine, propylthiouracil, and hydralazine have been reported 2…”