Abstract: Objective To identify the clinical features of adult patients with autoimmune enteropathy (AIE) in Chinese. Methods A respective study was conducted with the clinical data of 5 patients with adult AIE in Peking Union Medical College Hospital from 2011 to 2018, including clinical, endoscopic, histologic manifestations, medications and prognosis. Results Among the five patients, four were females, one was male, and their average age was 42 years old. The median disease duration was 2 months. The median hospitalization duration was 56 days. All patient initially presented with refractory diarrhea, with electrolyte disturbance, severe malnutrition, and slight elevation of inflammatory markers. 2 patients had hypovolemic shock and another 2 patients had Wernicke encephalopathy. Abdominal computed tomography findings including bowel-wall thickening, abnormal hyperenhancement of mucosal swelling, luminal dilatation, prominent mesenteric and retroperitoneal lymph nodes were nonspecific for AIE. Endoscopic appearances were mildly abnormal, including mucosal swellings and focal erosions. Specific histological features included villous blunting, absence of goblet or Paneth cells from the epithelium, increased crypt apoptotic bodies, lamina propria mononuclear infiltration, and surface intraepithelial lymphocytosis. Conclusions Clinical presentations and laboratory abnormalities of adult AIE are non-specific with minor endoscopic lesions. Therefore, Endoscopic biopsy, especially duodenal biopsy was important and indispensable in patients with suspected adult AIE.

Key words: Adult autoimmune enteropathy, Clinical features, Endoscopic manifestations, Histologic features