Autoimmune enteropathy: a review and update of clinical management.
Autoimmune enteropathy (AIE) is a rare condition characterized by intractable diarrhea, histologic changes on small intestinal biopsy, and failed response to dietary manipulation that also may present with extraintestinal manifestations. In many patients, immunosuppressive therapies are necessary. Although AIE is more common in infants, adult involvement has also been documented. Much of what is known about AIE has been gathered from case reports and small case series; therefore, more research in this evolving field is needed. IPEX (immunodysregulation polyendocrinopathy enteropathy X-linked syndrome) and APECED (autoimmune phenomena, polyendocrinopathy, candidiasis, and ectodermal dystrophy) are systemic forms of AIE.
Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA. gentile.nicole@mayo.edu
Nicole M
NM
Gentile
Joseph A
JA
Murray
Darrell S
DS
Pardi
D000328:Adult; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D007410:Intestinal Diseases / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D010288:Parenteral Nutrition
D007166:Immunosuppressive Agents