INTRODUCTION: Autoimmune Enteropathy (AIE) is a rare condition without established treatment guidelines. Typical symptoms include intractable diarrhea unresponsive to dietary changes, malabsorption, and weight loss. Common pathological findings include villous atrophy, crypt apoptotic bodies, lack of intraepithelial lymphocytes, and loss of goblet and Paneth cells. Anti-enterocyte and anti-goblet cell antibodies are found in 60-100% and 40%, respectively. Corticosteroids are first line treatment followed by immunosuppressive therapy. Total parenteral nutrition (TPN) is required in severe malnutrition. We have a case demonstrating the role of TNF therapy in steroid refractory AIE. CASE DESCRIPTION/METHODS: This patient is a 46 year old man with hypertension, hyperlipidemia, and atopic dermatitis who presented with severe diarrhea following lumbar fusion surgery, endorsing 20-30 Bristol 7 bowel movements daily. He was on a pain control regimen but reported taking minimal NSAIDs. Patient was not on an angiotensin receptor blocker. He presented from his primary care provider after he was found to have a potassium of 2.0 mEq/L. His nutritional status and electrolyte abnormalities continued to deteriorate and TPN was initiated. An MR enterography was performed and found no structural abnormalities of the small bowel. He underwent endoscopic evaluation where biopsies were taken from the duodenum, jejunum, ileum, and colon. Pathology reports showed crypt apoptosis and loss of both goblet and Paneth cells diagnostic for AIE. The patient started a high-dose steroid regimen of 60 mg prednisone daily with no significant clinical response after two weeks of therapy. Infliximab was started at 10 mg/kg every 8 weeks following a loading dose of 10 mg/kg at weeks 0, 2, and 6. Since beginning infliximab, the patient has experienced an improvement in symptoms and is currently at his baseline of 1–2 bowel movements per day. DISCUSSION: AIE is a rare cause of intractable diarrhea. Incidence is estimated at less than 1 in 100,000. Treatment consists of steroids, immunosuppressive agents, and nutritional support. Immunosuppressants that have been studied include infliximab, adalimumab, vedolizumab, and tacrolimus. Our case demonstrates the utility of infliximab for induction of remission and maintenance therapy of Autoimmune Enteropathy.
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