Primary autoimmune enteropathy

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Primary autoimmune enteropathy

A rare intestinal disease characterized by immune-mediated injury of the intestinal mucosa leading to severe chronic intractable diarrhea malabsorption and severe weight loss or failure to thrive. Characteristic histologic findings in the small intestine include partial or complete blunting of the villi deep crypt lymphocytosis increased crypt apoptosis and minimal surface intraepithelial lymphocytosis. In addition the stomach colon and esophagus may also be involved. Circulating autoantibodies against enterocytes and/or goblet cells are found in many but not all patients. The diagnosis requires exclusion of other causes of villous atrophy.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: // Data version October 2023.

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