Isolated Intestine Transplantation for Autoimmune Enteropathy: A Case Report

Autoimmune enteropathy is a rare disease characterized by chronic watery diarrhea, weight loss, and immune-mediated injury of the enterocolic mucosa. The clinicopathologic findings of this disease are variable, and timely diagnosis is challenging. It is usually managed medically. If medical management fails, surgical intervention is considered. This is a case report of a patient with autoimmune enteropathy mimicking collagenous enterocolitis. A 55-year-old man developed intestinal failure that manifested as profuse watery diarrhea, electrolyte disturbances, and weight loss. Initially, he was diagnosed with collagenous enterocolitis based on pathologic findings. Medical interventions were started, but the patient failed to show improvement. At 13 months after the onset of the disease, he was listed for isolated intestine transplantation (IITX) for intestinal failure. A healthy donor graft became available. IITX with chimney colostomy was performed. Based on the pathologic findings of the excised native small intestine, the patient was diagnosed with severe autoimmune enteropathy. The postoperative course was uneventful. By the third postoperative week, a full diet was tolerated and parenteral nutrition (PN) was weaned to end. He was discharged on postoperative day 34. Since discharge, he has been off PN, remaining on an enteral diet. This case is the first reported IITX performed on a patient with severe autoimmune enteropathy that was both curative and lifesaving. The present case confirms that IITX promptly restores gastrointestinal absorption in medically refractory autoimmune enteropathy. This observation provides clinicians with an effective treatment option in this challenging group of patients.