Small bowel (small intestine)
Inflammatory disorders
Autoimmune enteropathy


Topic Completed: 1 August 2012

Revised: 11 March 2019

Copyright: 2003-2019, PathologyOutlines.com, Inc.

PubMed Search: Autoimmune enteropathy small bowel

Hanni Gulwani, M.B.B.S.
Page views in 2018: 1,070
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Cite this page: Gulwani H. Autoimmune enteropathy. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/smallbowelautoimmuneenter.html. Accessed September 22nd, 2019.
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
Treatment
  • 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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