Autoimmune Enteropathy in Early Infancy: A Clinicopathological andImmunological Survey

Overview

This paper describes a severely affectedmale infant with serious protracted diarrhoea caused by a rare autoimmuneenteropathy. The disease began at 6 weeks of age of the child and it was associated with small bowel villous atrophyand the presence of circulating antienterocyte antibodies. The child was treated with steroids and with parenteraland special enteral nutrition. The patient showed clinical improvement as documented by decreased stool output andpossibility to terminate the parenteral nutrition. The small biopsy samples showed a return to normal. Antienterocyteantibodies were negative after the treatment. The patient has been followed up for at least 18 months and was ina clinical remission. We recommend that autoantibodies tests should be performed in all infants with unexplainedprotracted diarrhoea. The use of potent immunosuppressive drugs and the increasing experience with parenteral andenteral nutrition can improve the perspective of these previously fatal disorders.

Key words:
autoimmune enteropathy, antienterocyte antibodies, villous atrophy, immunosuppression, parenteralnutrition, infant.