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• W2922145438 abstract "Purpose: 67-year-old white man with past medical history significant for type II diabetes mellitus and COPD who presented with severe large volume watery diarrhea for 3 weeks prior to presentation. On examination; patient had signs of dehydration, abdominal examination showed soft, non tender abdomen. Extensive work up failed to reveal the underlying etiology. Fecal white blood cells were positive, otherwise, no evidence of infections or malabsorption. NPO status for 48 hours failed to correct the severe diarrhea. EGD and colonoscopy were performed which were normal. Random biopsies obtained from the small intestine and colon showed increased plasma cells and lymphocytes in the lamina propria,cryptitis and crypts abscesses formation, apoptosis and almost absence of goblet cells. Small intestine mucosa showed preserved villi. Anti- goblet cell enteropathy was suspected, serum anti-enterocytes antibodies sent to lab before starting patient on high dose prednisone which resulted in dramatic improvement. Subsequently patient was discharged home, after starting him on Azathioprine. Patient was followed multiple times in outpatient clinic with attempts to taper him off steroids, however, rapid taper of more than 5 mg of prednisone every 2 weeks resulted in recurrence in his diarrhea symptoms. Very slow taper was done with good success while increasing azathioprine to 2 mg/kg/day. Patient did well for few months till he presented with severe hypotension and signs of adrenal insuffieciency that required treatment in the intensive care unit. Subsequent work up revealed that patient had developed primary adrenal insufficiency, and he had to be maintained on hydrocortisone. All autoimmune markers were negative. HIV serology was negative. The initial anti-enterocyte antibodies sample was lost, subsequent samples while patient on immunesuppression were negative. Repeat biopsies from small intestine and colon showed reappearance of goblet cells with improvement in plasma cells infiltrate and colonic apoptosis. Discussion: Autoimmune enteropahty (AE) is a rare cause of chronic diarrhea in adults with few cases reported in the literature.The cornerstone of the diagnosis is small intestine biopsies that might resemble celiac disease, however, AE is a disorder that can span the whole GI tract and should be considered in refractory celiac disease patients. AE can be associated with or precede the development of autoimmune endocrionpathies like DM Type I, Hypothyrodism and addison's disease. Once the diagnosis of AE is made, it is very important to anticipate the possible development of these disorders which will facilitate early recognition and treatment which proved to be vital in our patient." @default.
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• W2922145438 date "2010-10-01" @default.
• W2922145438 modified "2023-09-25" @default.
• W2922145438 title "Autoimmune Enteropathy: A Rare Entity that Might Herald Autoimmune Endocrinopathies" @default.
• W2922145438 doi "https://doi.org/10.14309/00000434-201010001-00706" @default.
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