*Gastrointestinal Unit Departments of †Pathology and Laboratory Medicine ‡Rheumatology, Allergy and Immunology Division, Massachusetts General Hospital, Boston, MA.
J Clin Gastroenterol. 2014 Jan;48(1):55-8. doi: 10.1097/MCG.0b013e3182a4e0ec.
Autoimmune enteropathy (AIE) is a rare disease that has been observed in both children and adults. It typically manifests with symptoms of diarrhea, requiring long-term immunosuppression. Endoscopically, the duodenum typically exhibits villous blunting with partial or complete villous blunting, deep crypt lymphocytosis, increased apoptotic bodies, and minimal intraepithelial lymphocytosis on histologic analysis. The pathophysiology of AIE likely involves a hyperactive immune state in the setting of a T-cell regulatory defect, resulting in destruction of the enterocyte. We report a case of a 49-year-old woman who presented with refractory diarrhea, diagnosed as AIE. After failing multiple conventional therapies, she demonstrated clinical and histologic response to abatacept, a selective modulator of T-cell activation. We aim to increase awareness of this rare inflammatory disorder and new treatment options for this debilitating condition.
自身免疫性肠炎（AIE）是一种罕见的疾病，可发生于儿童及成人。其典型表现为腹泻症状，需要长期免疫抑制治疗。内镜下，十二指肠通常表现为绒毛变钝，部分或全部绒毛变钝，隐窝内淋巴细胞增多，凋亡小体增加，组织学分析显示上皮内淋巴细胞很少。AIE 的病理生理学可能涉及 T 细胞调节缺陷背景下的过度活跃免疫状态，导致肠上皮细胞破坏。我们报告了一例 49 岁女性，因难治性腹泻就诊，诊断为 AIE。在多次常规治疗失败后，她对 T 细胞活化选择性调节剂阿巴西普显示出临床和组织学应答。我们旨在提高对这种罕见炎症性疾病的认识，并为这种使人虚弱的疾病提供新的治疗选择。
