成人自身免疫性肠病三例及文献复习

Adult autoimmune enteropathy：Three cases report and literature review

目的明确成人自身免疫性肠病（AIE）临床诊断标准，探讨相关治疗方法。方法对我院过去3年中3例持续性严重腹泻且对禁食无效患者的病史进行回顾性分析。结果3名患者符合AIE描述的典型腹部症状并伴有严重低白蛋白血症，并排除其他自身免疫性疾病。病变部位限于十二指肠至回肠，病理检查示肠黏膜重姨慢性活动性炎症，绒毛结构消失。1例尝试性使用大剂量皮质激素后症状迅速改善，另2例在激素减量后出现腹泻症状反复，予甲氨蝶呤后好转。结论据此认为3名患者罹患罕见的成人自身免疫性肠病，建议有上述病症且禁食无效的患者应行肠黏膜活检，检测上皮细胞自身抗体及进行胶囊胃镜检查。

Objective To definitude the diagnostic standard and discuss the clinical trial strategies of autoimmune enteropathy （ALE）. Methods All three cases with intractable severe diarrhea and unresponsive to any dietary restrictions, hospitalized in Renji Hospital in the last three years , were collected and analyzed retrospectively. Results Three patients fulfilled the criteria of autoimmune enteropathy with the typical abdominal symptom, severe hypoalbuminemia and exclusion diagnosis of other autoimmune diseases. The lesions were located between duodenun and ileum with the pathological profile of chronic inflammation and villus atrophy in intestinal mucosa. One patient was impoved with large dose of steroid treatment ,while the other 2 patients got improvement at the beginning but relapsed after the dose of steroid reduced. The clinical condition of the two patients was stable with additional introduction of immunomodulators（MTX）. Conclusion Hereby we confirm the diagnosis of AIE for three patients and suggest those patients with the symptom of severe diarrhea and unresponsive to any dietary restrictions should undergo intestinal mucosa biopsy and capsule endoscope screening, and also test the anti-enterocyte antibody （AE） and anti-gobletcyte antibody （AG）.