Small bowel (small intestine)
Inflammatory disorders
Autoimmune enteropathy

Topic Completed: 1 August 2012

Minor changes: 2 April 2020

Copyright: 2003-2019,, Inc.

PubMed Search: Autoimmune enteropathy small bowel

Hanni Gulwani, M.B.B.S.
Page views in 2019: 1,668
Page views in 2020 to date: 703
Cite this page: Gulwani H. Autoimmune enteropathy. website. Accessed May 30th, 2020.
Definition / general
  • Severe and intractable watery diarrhea syndrome in infants associated with antibodies to intestinal epithelial cells
  • Includes IPEX syndrome, an X linked recessive disorder due to mutations in FOXP3 gene (Mod Pathol 2009;22:95)
  • Usually requires total parenteral nutrition
  • Similar condition in adults associated with variable immunodeficiency and type I diabetes, rheumatoid arthritis, hemolytic anemia
  • Immunosuppressive agents
Microscopic (histologic) description
  • Variable villus abnormality, few intraepithelial lymphocytes
  • May have colitis, increased expression of MHC class II antigens
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