Al Khalidi Hisham, Kandel Gabor, Streutker Cathy J
Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada.
Hum Pathol. 2006 Mar;37(3):373-6. doi: 10.1016/j.humpath.2005.11.019.
Autoimmune enteropathy (AIE) is a relatively rare condition found most frequently in children. It presents with persistent watery diarrhea and malabsorption and may require total parenteral nutrition for nutritional support. Rare cases have been reported in adults. On histology, the small intestinal villi are flattened but lack the intraepithelial lymphocytosis of celiac disease. In children and rarely in adults, some cases are linked to the IPEX syndrome (Immune dysregulation, Polyendocrinopathy, Enteropathy, X-linked inheritance). We report a 21-year-old man who presented with chronic persistent diarrhea for 4 years. The duodenal biopsies showed villous blunting, chronic inflammation, and decreased to absent goblet cells, Paneth cells, and endocrine cells by histology and electron microscopy. These changes are consistent with an AIE with involvement of non-enterocyte populations. Pathologists must be aware of the possibility of AIE in adults and consider it in the differential diagnosis of duodenitis, intraepithelial lymphocytosis, and small bowel villous flattening.
自身免疫性肠病（AIE）是一种相对罕见的疾病，多见于儿童。其表现为持续性水样腹泻和吸收不良，可能需要全胃肠外营养以提供营养支持。成人中也有罕见病例报道。在组织学上，小肠绒毛变平，但缺乏乳糜泻的上皮内淋巴细胞增多现象。在儿童中，部分病例与IPEX综合征（免疫失调、多内分泌腺病、肠病、X连锁遗传）有关，成人中则极为罕见。我们报告了一名21岁男性，他有4年慢性持续性腹泻病史。十二指肠活检显示，通过组织学和电子显微镜检查可见绒毛变钝、慢性炎症，杯状细胞、潘氏细胞和内分泌细胞减少或缺失。这些变化符合累及非肠上皮细胞群体的AIE。病理学家必须意识到成人患AIE的可能性，并在十二指肠炎症、上皮内淋巴细胞增多和小肠绒毛变平的鉴别诊断中考虑该病。
