A Rare Case of Adult Autoimmune Enterpathy

Abstract

Case Report: A 72-year-old woman with no prior significant medical history was admitted following a prolonged course of nonbloody mucoid diarrhea resulting in hypovolemic and cardiogenic shock requiring pressure support and intra-aortic balloon pump. Over the preceding 8 weeks, she had had multiple admissions for dehydration and electrolyte derangements despite diet modification and mineral supplements. Evaluation included negative stool studies including culture, ova/parasites, Cryptosporidium, Giardia, and Clostridium difficile. CT imaging of the abdomen and esophagogastroduodenoscopy (EGD) demonstrated duodenitis, but no evidence of celiac disease based on biopsy. She had been treated for small bowel bacterial overgrowth with metronidazole and empirically with anti-diarrheals without a notable improvement. Subsequent imaging was notable for diffuse mucosal edema in the setting of severe hypoabluminemia. Repeat EGD with duodenal biopsy demonstrated lymphocyte-rich infiltrate with reactive changes to the intestinal crypts including focal apoptosis of the crypt lining cells consistent with autoimmune enteritis. Anti-enterocyte antibody was negative. The patient was supported with total parenteral nutrition (TPN) and low-volume tube feeds, and started on 125 mg methylprednisolone. Nine days after initiation of therapy, follow-up EGD demonstrated improvement in inflammation.Figure 1: H&E stain of duodenum prior to steroid therapy. Evidence of direct damage to the intestinal crypts, including reactive changes, focal apoptosis of the crypt lining cells with intraepithelial lymphocytes, and crypt dropout.Conclusion: Autoimmune enteropathy is a rare disorder that presents predominantly in children, with only 30 cases reported in adults to date. It is thought to be a T-cell mediated process with increased crypt apoptotic bodies, minimal intraepithelial lymphocytosis, and villous blunting. Antienterocyte or anti-goblet cell antibodies support the diagnosis, but are not confirmatory. Previous case studies have shown complete response in up to 60% of patients treated with glucocorticoids and biologics for those who are refractory. However, there is no established standard therapy. Severe cases can lead to significant complications, particularly in the elderly population, if definitive care is not instituted early.