title: 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.
authors.affiliation: Institute of Internal Medicine, Catholic University, Rome, Italy. mmontalto@rm.unicatt.it
authors.forename: Massimo
authors.initials: M
authors.lastname: Montalto
authors.forename: Ferruccio
authors.initials: F
authors.lastname: D'Onofrio
authors.forename: Luca
authors.initials: L
authors.lastname: Santoro
authors.forename: Antonella
authors.initials: A
authors.lastname: Gallo
authors.forename: Antonio
authors.initials: A
authors.lastname: Gasbarrini
authors.forename: Giovanni
authors.initials: G
authors.lastname: Gasbarrini
mesh_terms: D000328:Adult; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000276:immunology* / Q000503:physiopathology / Q000628:therapy*; D002648:Child; D003967:Diarrhea; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008286:Malabsorption Syndromes / Q000175:diagnosis / Q000276:immunology* / Q000503:physiopathology / Q000628:therapy*; D018529:Nutritional Support; D011379:Prognosis; D015431:Weight Loss
chemical_list: D007166:Immunosuppressive Agents