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Autoimmune enteropathy and nephropathy with circulating anti-epithelial cell antibodies
R B Colletti1, A P Guillot, S Rosen
1Combined Program in Gastroenterology and Nutrition, Children's Hospital, Boston, Massachusetts.
Insights
Autoantibodies targeting epithelial cells were found in a child with autoimmune enteropathy and kidney disease. These antibodies correlated with disease activity, suggesting a potential role in the disorder.
Area of Science:
- Immunology
- Pediatric Gastroenterology
- Nephrology
Background:
- Autoimmune enteropathy and nephropathy can present with overlapping symptoms.
- The role of specific autoantibodies in pediatric autoimmune disorders requires further elucidation.
Observation:
- A child presented with persistent diarrhea, villous atrophy, and nephrotic syndrome due to membranous glomerulonephritis.
- Circulating antibodies against renal and intestinal epithelial cells were detected.
- A 55-kd antigen in both the small bowel and kidney reacted with the patient's serum antibodies.
Findings:
- High-dose prednisone initially induced remission, with relapse upon dose reduction, correlating with antibody levels and villous atrophy.
- Autoantibodies targeting a 55-kd epithelial cell protein were temporally linked to the development of enteropathy and nephropathy.
- Treatment resistance was observed later in the disease course.
Implications:
- Autoantibodies to a 55-kd epithelial cell protein may play a significant role in this specific autoimmune condition.
- Further investigation in similar patients is warranted to confirm the pathogenic role of these autoantibodies.
- Understanding this autoimmune mechanism could lead to targeted therapies for enteropathy and nephropathy.
Abstract:
We describe a child with circulating anti-epithelial cell antibodies, autoimmune enteropathy with intestinal villous atrophy, and membranous glomerulonephritis. The patient had persistent diarrhea at 6 months of age, and a small bowel biopsy showed active enteritis, villous atrophy, and crypt hyperplasia. When the patient was, 10 months of age, nephrotic syndrome developed because of membranous glomerulonephritis. Results of tests for circulating immune complexes were negative. Indirect immunofluorescence studies revealed a circulating antibody directed against renal epithelial cells. Circulating antibodies directed against normal small intestine epithelial cells were also detected by the immunoperoxidase technique. Western blot and immunoprecipitation identified a 55-kd antigen, in both small bowel and kidney, that reacted with an antibody in the patient's serum. High-dose prednisone therapy induced a clinical remission, resolution of the small bowel injury, and diminished serum anti-epithelial cell antibodies; after dose reduction, clinical relapse occurred with villous atrophy and reappearance of anti-epithelial cell antibodies. When the patient was 45 months of age, persistent diarrhea recurred despite intravenous administration of corticosteroids, cyclosporine, and total parenteral nutrition. Autoantibodies to a 55-kd epithelial cell protein are temporally related to the development of enteropathy and nephropathy. Study of similar patients is needed to determine the role of such antibodies in this disorder.