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Small bowel (small intestine)

Inflammatory disorders

Autoimmune enteropathy


Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 15 December 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● 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
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● Immunosuppressive agents

Micro description
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● Variable villus abnormality, few intraepithelial lymphocytes
● May have colitis, increased expression of MHC class II antigens

Micro images
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IPEX syndrome

End of Small bowel (small intestine) > Inflammatory disorders > Autoimmune enteropathy


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