Current Gastroenterology Reports

, Volume 14, Issue 5, pp 380–385 | Cite as

Autoimmune Enteropathy: A Review and Update of Clinical Management

  • Nicole M. Gentile
  • Joseph A. Murray
  • Darrell S. Pardi
Small Intestine (J Sellin, Section Editor)

Abstract

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.

Keywords

Autoimmune enteropathy (AIE) Immunodysregulation polyendocrinopathy enteropathy X-linked syndrome (IPEX) Autoimmune phenomena polyendocrinopathy candidiasis and ectodermal dystrophy (APECED) Intractable diarrhea 

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Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Nicole M. Gentile
    • 1
    • 2
  • Joseph A. Murray
    • 1
    • 3
  • Darrell S. Pardi
    • 1
  1. 1.Division of Gastroenterology and HepatologyMayo Clinic College of MedicineRochesterUSA
  2. 2.Department of Internal MedicineMayo Clinic College of MedicineRochesterUSA
  3. 3.Division of Gastroenterology and HepatologyMayo Clinic College of MedicineRochesterUSA

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