Autoimmune Enteropathy
Definition
- Gastrointestinal disease caused by autoimmune attack
- (Autoimmune atrophic gastritis is covered separately)
Alternate / Historical Names
- Generalized autoimmune gut disorder
See also
- IPEX syndrome(Immune Dysregulation, Polyendocrinopathy, Autoimmune Enteropathy, X-linked)
Diagnostic Criteria
- Autoimmune enteritis usually presents in first six months of life
- Principally involves small intestine
- May also involve stomach and large intestine
- Nearly all show partial or complete villous atrophy
- Nearly all show an increase in lamina propria lymphocytes
- Infiltrate predominantly composed of CD3+ CD4+ alpha beta T cells
- Mild to marked increase in intra-epithelial lymphocytes
- Variable reports in the literature
- Infiltrate predominantly composed of CD3+ CD4+ alpha beta T cells
- Inconspicuous to prominent apoptosis in crypts
- Crypts may be hyperplastic or destroyed
- Goblet cells may be absent
- Anti-enterocyte brush border antibodies demonstrable by indirect immunofluorescence
- Anti-goblet cell antibodies may be present but less specific
- May occur in at least three forms
(Ruemmele 2008)
- Associated with autoimmune endocrinopathy and mutations in FOXP3 gene leading to X-linkage, see IPEX syndrome
- Same associations as IPEX syndrome except no X-linkage
- Isolated autoimmune enteritis
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Original posting/updates : 11/11/09, 11/20/11
