Autoimmune enteropathy

Autoimmune enteropathy, abbreviated AIE, is a rare gastrointestinal pathology.

General

• Considered a pediatric disease.
• Super rare in adults - there are only ~11 reported cases in the literature.
• May be a component of the IPEX syndrome.

Diagnosis is clinico-pathologic:

1. Intact immune system.
2. Autoantibodies.
   • Anti-enterocyte antibody.
   • Anti-goblet antibody.
3. Lack of response to gluten-free diet.

Microscopic

Features:

• +/-Loss of goblet cells.
• +/-Loss of paneth cells.
• Villous blunting.

DDx:

• Celiac disease.

See also

• Small intestine.