Autoimmune enteropathy type 3 (Q104377): Difference between revisions

@@ description / pt-br / description / pt-br @@
+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.
@@ Property / Canonical URI @@
+https://id.who.int/icd/entity/1137579941
@@ Property / Canonical URI: https://id.who.int/icd/entity/1137579941 / rank @@
+Normal rank
@@ Property / CURIE @@
+CID11:ID_1137579941
@@ Property / CURIE: CID11:ID_1137579941 / rank @@
+Normal rank
@@ Property / Canary Token @@
+dki-india-ID_1137579941
@@ Property / Canary Token: dki-india-ID_1137579941 / rank @@
+Normal rank
@@ Property / Verification Status @@
+Concluído
@@ Property / Verification Status: Concluído / rank @@
+Normal rank
@@ Property / Knowledge Architect @@
+https://pauloleads.com.br/cases-publicos/
+Normal rank
@@ Property / Collection date @@
+August 2026Timestamp +2026-08-16T00:00:00Z
Timezone +00:00
Calendar Gregorian
Precision 1 day
Before 0
After 0
-Timestamp
++2026-08-16T00:00:00Z
-Timezone
++00:00
-Calendar
+Gregorian
-Precision
+day
 Before
 After
@@ Property / Collection date: 16 August 2026 / rank @@
+Normal rank

Latest 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
default for all languages	ID_1137579941
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.