autoimmune enteropathy

autoimmune enteropathy

A disorder of the small intestine featuring severe atrophy of the absorptive VILLI and circulating antibodies against the cells that form the lining of the intestine (enterocytes). The condition does not respond to a gluten-free diet but there is a good response if this is combined with treatment directed against the immune system process that produces the enterocyte autoantibodies.
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Autoimmune enteropathy is a rare condition characterized by immune-mediated intestinal mucosal atrophy.
Autoimmune enteropathy is characterized by refractory diarrhea with malabsorption, histologic changes on small intestinal biopsy, and the need for immunosuppressive treatment [15].
Autoimmunity findings, which may be observed in IPEX, include type 1 DM, thyroiditis, autoimmune cytopenias, eczema, autoimmune enteropathy, hepatitis, nephrotic syndrome, interstitial nephritis, and arthritis (11).
It is a unique T-cell autoimmune enteropathy that is precipitated in genetically susceptible individuals by the ingestion of gluten, the major storage protein of wheat, barley, and rye.
Celiac disease is an autoimmune enteropathy triggered by gluten ingestion for which a gluten-free diet is currently the sole treatment.
Celiac disease (CD) is a chronic autoimmune enteropathy occurring in genetically predisposed individuals following ingestion of wheat gluten and related protein fractions of other grains.
When follow-up endoscopy reveals persisting villous atrophy, histological evaluation should focus on identifying other causes of villous atrophy such as Giardiasis, immunodeficiencies, collagenous sprue, Whipple's disease, and autoimmune enteropathy [18].
Autoimmune enteropathy is often found in patients with selective IgA deficiency, common variable immunodeficiency (CVID) and IPEX,3,4 but the role of NK cells remains undetermined.6,7 In our case report, patient initially presented with CD which was difficult to manage along with recurrent, opportunistic infections such as candidiasis and CMV disease.
Gary and Ross were born with life-limiting gastrointestinal disease autoimmune enteropathy. It causes the body's immune system to attack its intestinal wall, causing chronic diarrhoea.
Five criteria for diagnosing autoimmune enteropathy are now available thanks to a compilation of 15 patients at the Mayo Clinic, which has more than doubled the number of cases in the published literature.
Celiac disease is an autoimmune enteropathy characterized by an inappropriate T-cell--mediated reaction to eating gluten and similar prolamines found in wheat, rye, and barley in genetically susceptible individuals.

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