저자들은 만성 분비성 설사와 성장부진을 주소로 내원한 10개월된 남아에서 소장생검조직소견과 Western blot과 면역조직화학검사로 장상피세포에 대한 자가항체를 확인함으로써 자가면역성 장병증으로 진단하고, 코르티코스테로이드와 FK506으로 성공적으로 치료한 1례를 경험하였기에 이를 보고하는 바이다.
Autoimmune enteropathy is a rare chronic diarrheal disease of infancy. Clinicopathologically, this entity is characterized by chronic secretory diarrhea, villous atrophy with crypt hypoplasia of a small intestine and/or associated autoimmune disorders, and absence of severe immunodeficiency. For the confirmation of diagnosis, antienterocyte autoantibody should be delineated. The treatment of choice of this disorder is immunosuppression. We has been experienced a case of autoimmune enteropathy without autoimmune disorders in a 10-month-old male infant. He developed protracted diarrhea from 5 months of his age and has been appeared to be failure to thrive. Antienterocyte autoantibody was demonstrated by immunohistochemistry and western blotting. He was successfully treated with corticosteroid and FK506. This is the first case report of autoimmune enteropathy without autoimmune disorders in Korea.
