Autoimmune enteropathy type 3 (Q104377): Difference between revisions
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Enteropatia autoimune (AIE) é uma condição rara caracterizada por diarreia intratável, alterações histológicas na biópsia do intestino delgado, falha na resposta à manipulação dietética que também pode apresentar manifestações extraintestinais. Em muitos pacientes, terapias imunossupressoras são necessárias. Embora a AIE seja mais comum em bebês, o envolvimento em adultos também foi documentado. IPEX (síndrome de desregulaçao imune, poliendocrinopatia e enteropatia ligada ao X) ou APECED (Fenômenos Autoimunes, Poliendocrinopatia, Candidíase e Distrofia Ectodérmica) são formas sistêmicas de AIE. | |||
| description / en | description / en | ||
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. | |||
Revision as of 10:14, 17 August 2026
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 |
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| 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. |
