Autoimmune enteropathy can result in diarrhea and malabsorption.


Clinical features:

(1) chronic diarrhea, often starting during infancy

(2) malabsorption

(3) often extraintestinal autoimmune disorders (hemolytic anemia, immune thrombocytopenia, Sjogren's syndrome, polyarthritis, diabetes)

(4) family history of unexplained diarrhea during infancy

(5) clinical improvement with immunosuppression


Histologic features:

(1) villus atrophy

(2) dense lymphocytic infiltrate composed of T cells


Laboratory findings - one of the following in the serum:

(1) autoantibody against enterocytes

(2) autoantibody against goblet cells



(1) primary immune deficiency

(2) microvillus inclusion disease

(3) tufting enteropathy

(4) celiac disease


Favorable Prognosis

Poor Prognosis

daily stool output

< 80 mL per kg

> 150 mL per kg

crypt damage


crypt abscesses or necrosis

other digestive lesions

none or mild nonspecific colitis

extension of lymphoid infiltrate into colon


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