Virchows Archiv

, Volume 472, Issue 1, pp 55–66 | Cite as

Autoimmune enteropathies

  • Sarah E. UmetsuEmail author
  • Ian Brown
  • Cord Langner
  • Gregory Y. Lauwers
Invited Annual Review Issue


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.


Autoimmune disease Immunodeficiency Drug induced Inherited disease Enteritis Colitis 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© Springer-Verlag GmbH Deutschland 2017

Authors and Affiliations

  • Sarah E. Umetsu
    • 1
    Email author
  • Ian Brown
    • 2
  • Cord Langner
    • 3
  • Gregory Y. Lauwers
    • 4
  1. 1.Department of PathologyUniversity of California, San FranciscoSan FranciscoUSA
  2. 2.Envoi PathologyKelvin GroveAustralia
  3. 3.Institute of PathologyMedical University of GrazGrazAustria
  4. 4.Department of PathologyMoffitt Cancer CenterTampaUSA

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