Methylprednisolone pulse therapy and intravenous cyclophosphamide therapy combined with cocktail therapy in severe pediatric Henoch-Schönlein purpura nephritis patient.

CEN Case Reports
Hiroaki KanaiKosuke Higashida

Abstract

Henoch-Schönlein purpura (HSP) is a common self-limited vasculitis in children. The long-term prognosis depends on renal involvement. In severe Henoch-Schönlein purpura nephritis (HSPN) patients, >50 % have crescent formation and nephrotic syndrome that are important predicted outcomes. Therefore, for such patients, an aggressive immunosuppressive therapy is needed to prevent the progression. However, there is no consensus for an appropriate therapeutic regimen for severe pediatric HSPN patients. In this paper, we have reported on a 6-year-old boy who presented with HSPN with nephrotic syndrome and severe histopathological abnormalities; he was diagnosed with International Study of Kidney Disease in Children (ISKDC) grade IVb. Despite treatment with methylprednisolone pulse therapy, followed by oral prednisolone and dipyridamole; the nephrotic syndrome persisted. Subsequently, intravenous cyclophosphamide therapy (IVCY) (500-1,000 mg m-2 once a month for 7 months; total 6,000 mg m-2) was administered, followed by azathioprine and enarapril. Within 7 months of disease onset, complete remission was achieved. After 22 months of the initial renal biopsy, the second biopsy was performed to confirm treatment efficacy. Histopathologic...Continue Reading

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Citations

Jul 31, 2021·Jornal brasileiro de nefrologia : ʹorgão oficial de Sociedades Brasileira e Latino-Americana de Nefrologia·Mehtap Akbalik KaraAyşe Balat

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Methods Mentioned

BETA
light microscopy
biopsy

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