Catalog

Autoimmune enteropathy type 2

No MMS code

Link to WHO Definition

Autoimmune enteropathy type 2 – http://id.who.int/icd/entity/1341117913

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.