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Published in:

01-01-2018 | Invited Annual Review Issue

Autoimmune enteropathies

Authors: Sarah E. Umetsu, Ian Brown, Cord Langner, Gregory Y. Lauwers

Published in: Virchows Archiv | Issue 1/2018

Abstract

Autoimmune enteropathy (AIE) is a rare condition characterized by intractable diarrhea and immune-mediated injury of the intestinal mucosa. As the clinical and histopathologic manifestations of this disease are highly variable, its diagnosis is challenging for both clinicians and pathologists. In fact, the term autoimmune enteropathies is likely more appropriate since the clinicopathologic manifestations are observed in association with a heterogeneous group of disorders. The pathophysiology of AIE has not been fully elucidated. It appears to result from dysregulation of intestinal immunity and particularly in children, often presents in association with immunodeficiency. The overarching histopathologic changes seen in AIE include mucosal inflammation and epithelial injury, although this can manifest in the form of different patterns. Recognition of the clinical settings and of the various histologic patterns can aid the pathologist in establishing the correct diagnosis.

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Title: Autoimmune enteropathies
Authors: Sarah E. Umetsu
Ian Brown
Cord Langner
Gregory Y. Lauwers
Publication date: 01-01-2018
Publisher: Springer Berlin Heidelberg
Published in: Virchows Archiv / Issue 1/2018
Print ISSN: 0945-6317
Electronic ISSN: 1432-2307
DOI: https://doi.org/10.1007/s00428-017-2243-7

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