Autoimmune enteropathy in children and adults.

Abstract:

:Autoimmune enteropathy is a rare disorder characterized by severe and protracted diarrhea, weight loss from malabsorption and immune-mediated damage to the intestinal mucosa, generally occurring in infants and young children, although some cases of adult onset have been reported in the literature. Pathogenetic mechanisms involve immunological disorders, in which the presence of antienterocyte autoantibodies, although detected since first description, seems now to be secondary. As occurs frequently in autoimmunity, subjects with autoimmune enteropathy may be affected by other autoimmune disorders, sometimes leading to particular forms, i.e. the IPEX syndrome and the APECED syndrome. The prognosis of autoimmune enteropathy patients depends on the severity of digestive symptoms (including fecal output), on the severity and extension of histological lesions along the gastrointestinal apparatus, and on the presence of extra-intestinal involvement. Management of autoimmune enteropathy patients is based on nutritional support and adequate hydration to ensure optimal growth and development, together with immunosuppressive therapy. Recently, biological agents have been introduced, with apparent beneficial effects.

journal_name

Scand J Gastroenterol

authors

Montalto M,D'Onofrio F,Santoro L,Gallo A,Gasbarrini A,Gasbarrini G

doi

10.1080/00365520902783691

subject

Has Abstract

pub_date

2009-01-01 00:00:00

pages

1029-36

issue

9

eissn

0036-5521

issn

1502-7708

pii

909181497

journal_volume

44

pub_type

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