Small bowel (small intestine)
Inflammatory disorders
Autoimmune enteropathy

Author: Hanni Gulwani, M.D. (see Authors page)

Revised: 12 February 2018, last major update August 2012

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Autoimmune enteropathy small bowel

Cite this page: Gulwani, H. Autoimmune enteropathy. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/smallbowelautoimmuneenter.html. Accessed July 18th, 2018.
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