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.
KeywordsAutoimmune 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. 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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