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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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).
Patients often receive a diagnosis and are treated for other causes of enteropathy before a diagnosis of OAE is given; these other causes are most commonly celiac sprue or autoimmune enteropathy.
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.
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.
Gary and Ross were born with life-limiting gastrointestinal disease autoimmune enteropathy.
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.
It is unlikely that what we are calling corticosteroidresponsive enteropathy of infancy represents a true autoimmune enteropathy because only four (27%) of 15 biopsied patients had severe intestinal villus atropy.
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.
However, other entities such as tropical sprue, autoimmune enteropathy (AE; illustrated in Figure 3, A and B), common variable immunodeficiency (CVID; illustrated in Figure 3, C and D), collagenous sprue (illustrated in Figure 4, A and B), and drug-induced mucosal injury (such as colchicine toxicity, illustrated in Figure 4, C), among other disorders listed in Table 3, can all cause villous atrophy, with or without a concomitant increase in IEL numbers.

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