Atypical Presentation of Autoimmune Enteropathy

Abstract

Purpose: Autoimmune enteropathy (AIE) is a disease which has been sporadically described in childhood and even more rarely in adults. Most cases of AIE described in adults are confined to young or middle-aged women and a high prevalence of other organ- and non-organ-specific autoantibodies with associated autoimmune manifestations. Case: We report the case of an elderly woman with AIE who presented with sudden onset nausea, vomiting and weight loss with no diarrhea. A 67-year-old woman presented with sudden onset nausea and post-prandial vomiting for a period of 6 months. This was not preceded by any viral illness and her only significant past medical history was hypothyroidism. She also developed severe weight loss of greater than 60 lbs over a period of 6 months. She did not report any diarrhea and her gastric emptying study was normal. Duodenal biopsy showed a variable villous abnormality associated with a mixed infiltrate within the lamina propria, including lymphocytes, plasma cells, and eosinophils. There was evidence of fairly prominent apoptosis within some of the crypts. However the patient had negative serology for celiac disease and no evidence of intra-epithelial lymphocytosis. The gastric biopsy also showed evidence of rare apoptotic cells. Further evaluation for autoimmune enteropathy was done and the work up was significant only for the presence of positive anti-nuclear antibodies in high titer (1:320). The serum immunoglobulin levels were normal. The overall clinical picture was suggestive of autoimmune enteropathy with no diarrhea. Treatment by steroids brought to the remission her symptoms of nausea and vomiting within 6 weeks. Conclusion: Autoimmune enteropathy should be always ruled out in patients with villous atrophy and autoimmune manifestations even in patients with atypical presentation like nausea and vomiting without diarrhea.