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
Exclusions:
(1) primary immune deficiency
(2) microvillus inclusion disease
(3) tufting enteropathy
(4) celiac disease
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Finding
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Favorable Prognosis
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Poor Prognosis
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daily stool output
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< 80 mL per kg
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> 150 mL per kg
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crypt damage
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none
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crypt abscesses or necrosis
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other digestive lesions
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none or mild nonspecific colitis
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extension of lymphoid infiltrate into colon
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