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Diffuse Autoimmune Enteropathy with Colitis in an Adult Patient Treated Successfully with Adalimumab


Hasan, Syed DO1; Siddiqui, Nauman MD1; Russo, Pierre MD2; Las Casas, Luis Des MD1; Nawras, Ali MD, FACG1

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American Journal of Gastroenterology: October 2013 - Volume 108 - Issue - p S290
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Purpose: Autoimmune enteropathy (AIE) is a rare disease that causes intractable diarrhea not responsive to a gluten free diet and must be distinguished from refractory sprue. It is associated with circulating autoantibodies against goblet cells and enterocytes. AIE mainly involves the small intestines and is most commonly found in children, with very few cases reported in adults. Due to the paucity of cases, the epidemiology of the disease remains unclear. This is the case of a 28-year-old female who presented due to diarrhea with 20-40 episodes of voluminous green liquid stool per day. Stool cultures for bacteria, viruses, ova and parasites were all negative as were workups for secretory diarrhea, neuroendocrine causes, and testing for celiac disease. The patient underwent endoscopic gastroduodenoscopy and colonoscopy, which revealed mild diffuse hyperemia with no ulcerations. Biopsies showed intensely destructive enteritis, far more than would be expected from infection, with crypt abscesses, reduced intraepithelial lymphocytes, and abundant apoptosis, characteristic for adult onset autoimmune enteropathy. The diagnosis was confirmed by positive staining of IgG along the apex of the erythrocytes and positive circulating anti-enterocyte antibodies. She was started on methylprednisolone, and after a few days, started to have more formed stool with gradually decreased frequency. She was started on adalimumab therapy with good response and control of her diarrhea. The lack of data regarding this disease and subsequent shortage of histologic expertise makes the diagnosis difficult to make. In addition to the fact that our patient developed adult-onset AIE, she was also noted to have intense inflammation in the colon. AIE is most often restricted to the small bowel but there have been a small number of case reports showing manifestations outside of the small bowel, mostly seen in children. To our knowledge, this is the first reported case of adult onset autoimmune enteropathy with colitis, successfully treated with adalimumab.

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