Autoimmune enteropathy type 3 (Q104377)
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Autoimmune Enteropathy (AIE) is a rare condition characterised by intractable diarrhoea, histologic changes on small intestinal biopsy, failed response to dietary manipulation that also may present with extra-intestinal manifestations. In many patients, immunosuppressive therapies are necessary. Although AIE is more common in infants, adult involvement has also been documented. IPEX (Immunodysregulation Polyendocrinopathy Enteropathy X-linked Syndrome) or APECED (Autoimmune Phenomena, Polyendocrinopathy, Candidiasis, and Ectodermal Dystrophy) are systemic forms of AIE.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | ID_1137579941 |
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| English | Autoimmune enteropathy type 3 |
Autoimmune Enteropathy (AIE) is a rare condition characterised by intractable diarrhoea, histologic changes on small intestinal biopsy, failed response to dietary manipulation that also may present with extra-intestinal manifestations. In many patients, immunosuppressive therapies are necessary. Although AIE is more common in infants, adult involvement has also been documented. IPEX (Immunodysregulation Polyendocrinopathy Enteropathy X-linked Syndrome) or APECED (Autoimmune Phenomena, Polyendocrinopathy, Candidiasis, and Ectodermal Dystrophy) are systemic forms of AIE. |
