Autoimmune Enteropathy in Early Infancy: A Clinicopathological and
Sýkora J., Varvařovská J., Stožický F. , Kobr J., Pizingerová K., 1Schejbalová E., 2Tláskalová H., 2Tučková L., 3Šedivá A., 4Janatková I., 5Gutová V.
Dětská klinika FN a LF UK, Plzeň 1Šiklův patologicko-anatomický ústav FN a LF UK, Plzeň 2Mikrobiologický ústav AV ČR, sektor imunologie a gnotobiologie, Praha 3Ústav imunologie 2. LF UK a FNM, Praha 4Ústav klinické imunologie a alergologie 1. LF UK a VFN, Praha 5Ústav klinické imunologie a alergologie FN a LF UK, Plzeň
This paper describes a severely affectedmale infant with serious protracted diarrhoea caused by a rare autoimmune
enteropathy. The disease began at 6 weeks of age of the child and it was associated with small bowel villous atrophy
and the presence of circulating antienterocyte antibodies. The child was treated with steroids and with parenteral
and special enteral nutrition. The patient showed clinical improvement as documented by decreased stool output and
possibility to terminate the parenteral nutrition. The small biopsy samples showed a return to normal. Antienterocyte
antibodies were negative after the treatment. The patient has been followed up for at least 18 months and was in
a clinical remission. We recommend that autoantibodies tests should be performed in all infants with unexplained
protracted diarrhoea. The use of potent immunosuppressive drugs and the increasing experience with parenteral and
enteral nutrition can improve the perspective of these previously fatal disorders.
autoimmune enteropathy, antienterocyte antibodies, villous atrophy, immunosuppression, parenteral