Japanese journal of pediatric nephrology
Online ISSN : 1881-3933
Print ISSN : 0915-2245
ISSN-L : 0915-2245

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Membranoproliferative-like IgA vasculitis nephritis in children: report of two cases
Takuji YamadaMasayoshi Yoshikane
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JOURNAL OPEN ACCESS Advance online publication

Article ID: cr.2021.0194

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Abstract

Kidney involvement in IgA vasculitis (IgAV) is the most serious complication and renal biopsy findings in patients are mainly graded according to the criteria of the International Study of Kidney Disease in Children (ISKDC). Case 1 was a 3-year-old girl presented with nephrotic syndrome 1 month after initial presentation with IgAV, in whom pathologic renal biopsy revealed membranoproliferative-like lesions. Urinary findings gradually improved treated with intravenous methylprednisolone (IVMP), followed by multiple-drug therapy. Case 2 was a 6-year-old boy who underwent renal biopsy for nephritic-nephrotic syndrome on the 3 weeks after the onset of IgAV. Pathologic findings showed membranoproliferative-like lesions, and tuft necrosis and cellular crescents in about half of the glomeruli. IVMP and plasma exchange, followed by multiple-drug therapy, were performed. At the time of relapse about 2 years later, re-biopsy showed only new active lesions, and additional treatment with IVMP led to clinical remission after 4 years after the start of treatment. Patients in IgAV nephritis of ISKDC grade 6, membranoproliferative-like lesions, have diverse clinical and pathological features, but not necessarily with a bad prognosis. Hence, it may be important to perform stratified treatment by reclassification and semiquantification based on clinical symptoms and histological findings at the time of onset.

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© 2021 The Japanese Society for Pediatric Nephrology

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https://creativecommons.org/licenses/by-nc-sa/4.0/deed.ja
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