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)


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.


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



This work was supported in part through NIH Grant DK57892.

Dr. Thomas C Smyrk, MD, Department of Pathology, Mayo Clinic, College of Medicine for providing Figs. 1 and 2.


Dr. J. Murray has received grant support from the NIH and Alba Therapeutics, and consulting fees or honoraria from Ironwood, Inc., Flamenteras, Actogenix, Bayer Healthcare, Vysera Biomedical, 2G Pharma, Inc., ImmunosanT, Inc., and Shire US Inc. No other potential conflicts of interest relevant to this article were reported.


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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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