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

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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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Authors and Affiliations

  1. Pediatric Nephrology, Children’s Hospital of Michigan, 3901 Beaubien, Detroit, MI, 48201, USA

    Shina Menon & Rudolph Valentini

  2. Department of Laboratory Medicine and Pathology, Wayne State University, Detroit, MI, USA

    Xu Zeng

Authors
  1. Shina Menon
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  2. Xu Zeng
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  3. Rudolph Valentini
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Correspondence to Shina Menon.

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Menon, S., Zeng, X. & Valentini, R. Fibrillary glomerulonephritis and renal failure in a child with systemic lupus erythematosus. Pediatr Nephrol 24, 1577–1581 (2009). https://doi.org/10.1007/s00467-009-1168-z

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  • DOI: https://doi.org/10.1007/s00467-009-1168-z

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