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Related Experiment Videos

[Renal involvement in autoimmune enteropathies]

R Habib1, A Beziau, O Goulet

  • 1INSERM U192, Hôpital Necker-Enfants Malades, Paris.

Annales De Pediatrie
|February 1, 1993
PubMed
Summary
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Autoimmune enteropathy in infants can lead to kidney disease, specifically membranous glomerulonephritis (MGN). This case highlights auto-antibodies targeting renal brush border proteins as a cause of MGN in autoimmune enteropathy.

Area of Science:

  • Nephrology
  • Immunology
  • Pediatric Gastroenterology

Background:

  • Autoimmune enteropathy is a rare condition characterized by chronic diarrhea, neonatal diabetes, and dermatitis.
  • Renal involvement, including nephrotic syndrome, can occur in autoimmune enteropathy.

Observation:

  • A 4-month-old infant with autoimmune enteropathy developed nephrotic syndrome.
  • Renal biopsy revealed membranous glomerulonephritis (MGN) with IgG linear deposits along tubular basement membranes.
  • The patient's serum contained anti-enterocyte, anti-tubular basement membrane (anti-TBM), and anti-renal brush border (anti-BB) antibodies.

Findings:

  • Immunosuppressive therapy was ineffective.
  • Anti-TBM antibodies decreased over time, while anti-BB antibodies persisted.

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  • Literature review shows MGN in autoimmune enteropathy is associated with anti-TBM or anti-BB antibodies.

    • Implications:

    • This case suggests that auto-antibodies to renal brush border (BB) proteins can cause MGN in humans.
    • A potential common epitope between renal BB proteins and gut epithelial proteins may explain the link.
    • Further research is needed to elucidate the immunopathogenesis of renal disease in autoimmune enteropathy.