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Autoimmune enteropathy (view source)
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# | '''Autoimmune enteropathy''', abbreviated '''AIE''', is a rare gastrointestinal pathology. | ||
==General== | |||
*Considered a pediatric disease. | |||
*Super rare in adults - there are only ~11 reported cases in the literature.<ref name=pmid17683994>{{Cite journal | last1 = Akram | first1 = S. | last2 = Murray | first2 = JA. | last3 = Pardi | first3 = DS. | last4 = Alexander | first4 = GL. | last5 = Schaffner | first5 = JA. | last6 = Russo | first6 = PA. | last7 = Abraham | first7 = SC. | title = Adult autoimmune enteropathy: Mayo Clinic Rochester experience. | journal = Clin Gastroenterol Hepatol | volume = 5 | issue = 11 | pages = 1282-90; quiz 1245 | month = Nov | year = 2007 | doi = 10.1016/j.cgh.2007.05.013 | PMID = 17683994 | PMC = 2128725 | URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2128725/ }}</ref> | |||
*May be a component of the IPEX syndrome.<ref>{{Cite journal | last1 = Gentile | first1 = NM. | last2 = Murray | first2 = JA. | last3 = Pardi | first3 = DS. | title = Autoimmune enteropathy: a review and update of clinical management. | journal = Curr Gastroenterol Rep | volume = 14 | issue = 5 | pages = 380-5 | month = Oct | year = 2012 | doi = 10.1007/s11894-012-0276-2 | PMID = 22810979 }}</ref> | |||
Diagnosis is clinico-pathologic:<ref name=pmid17683994/> | |||
#Intact immune system. | |||
#Autoantibodies. | |||
#*Anti-enterocyte antibody. | |||
#*Anti-goblet antibody. | |||
#Lack of response to gluten-free diet. | |||
==Microscopic== | |||
Features:<ref name=pmid17683994/> | |||
*+/-Loss of goblet cells. | |||
*+/-Loss of paneth cells. | |||
*Villous blunting. | |||
DDx: | |||
*[[Celiac disease]]. | |||
==See also== | |||
*[[Small intestine]]. | |||
==References== | |||
{{Reflist|1}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Gastrointestinal pathology]] | |||