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Published in: Pediatric Nephrology 8/2009

01-08-2009 | Brief Report

Fibrillary glomerulonephritis and renal failure in a child with systemic lupus erythematosus

Authors: Shina Menon, Xu Zeng, Rudolph Valentini

Published in: Pediatric Nephrology | Issue 8/2009

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Abstract

Fibrillary glomerulonephritis (FGN) is a rare immune-mediated glomerulopathy characterized by randomly arranged immunoglobulin (Ig) deposits on electron microscopy. Only seven pediatric cases have been reported, and the incidence in adults is about 1.5%. A 12-year-old boy presented with systemic lupus erythematosus (SLE) with World Health Organization Class IV lupus nephritis. A repeat biopsy carried out due to a poor response to standard immunosuppressive therapy and worsening renal functions revealed diffuse proliferative glomerulonephritis with fibrillary deposits. Despite aggressive immunosuppression with plasmapheresis and rituximab, the patient developed end stage renal disease. This is an atypical pediatric case characterized by SLE-associated FGN and a poor prognosis.
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Metadata
Title
Fibrillary glomerulonephritis and renal failure in a child with systemic lupus erythematosus
Authors
Shina Menon
Xu Zeng
Rudolph Valentini
Publication date
01-08-2009
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 8/2009
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-009-1168-z

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