Autoimmune enteropathy: A review and update of clinical management

Nicole M. Gentile, Joseph A. Murray, Darrell S. Pardi

Research output: Contribution to journalReview articlepeer-review

70 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)380-385
Number of pages6
JournalCurrent gastroenterology reports
Issue number5
StatePublished - Oct 2012


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

ASJC Scopus subject areas

  • Gastroenterology


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