Abatacept A New Treatment Option for Refractory Adult Autoimmune Enteropathy
Abatacept A New Treatment Option for Refractory Adult Autoimmune Enteropathy
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DOI：
10.1097/mcg.0b013e3182a4e0ec
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发表时间：
2014-01-01
影响因子：
2.9
通讯作者：
Yajnik, Vijay
中科院分区：
文献类型：
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作者：
Gupta, Nitin K.;Yilmaz, Omer;Yajnik, Vijay
自身免疫性肠病（AIE）是一种罕见的疾病，已观察到在儿童和成人。它通常表现为腹泻症状，需要长期免疫抑制。通过内窥镜检查，十二指肠通常表现出绒毛变钝，伴部分或完全绒毛变钝、深隐窝淋巴细胞增多、凋亡小体增加以及组织学分析显示极轻微的上皮内淋巴细胞增多。AIE的病理生理学可能涉及在T细胞调节缺陷的情况下的过度活跃的免疫状态，导致肠上皮细胞的破坏。我们报告一例49岁的女性谁提出了难治性腹泻，诊断为AIE。在多次常规治疗失败后，她表现出对阿巴西普（一种T细胞活化的选择性调节剂）的临床和组织学反应。我们的目标是提高人们对这种罕见的炎症性疾病的认识，并为这种使人衰弱的疾病提供新的治疗选择。
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.