中国成人自身免疫性肠病的临床特点分析

Clinical characteristics of Chinese patients with adult autoimmune enteropathy

目的通过分析中国成人自身免疫性肠病(AIE)的临床特点,提高对该疾病的认识和早期诊断水平。方法回顾性分析北京协和医院2011年至2018年收治的5例成人自身免疫性肠病患者的临床表现、影像学及内镜检查结果、病理特点及治疗随访情况。结果5例AIE患者中男1例,女4例,平均年龄42岁;中位病程2个月,中位住院日期56d;均以顽固性大量水样泻为首发症状,多伴有电解质紊乱、严重营养不良和炎性指标轻度升高;有2例出现低血容量性休克、2例并发Wernicke脑病。腹盆CT检查示非特异性肠道病变,可表现为肠壁增厚、肠黏膜强化、肠腔积液积气、腹膜后及肠系膜周围多发淋巴结形态饱满或轻度增大。内镜下病变轻微,可见黏膜充血肿胀、粗糙不平、点状糜烂灶。病理表现典型,包括小肠绒毛短缩、上皮内杯状细胞、潘氏细胞减少、隐窝凋亡小体增多、黏膜固有层炎细胞浸润、上皮内淋巴细胞增多。结论成人AIE的临床表现和辅助检查缺乏特异性。内镜下可仅有轻微病变,对疑诊AIE的患者应积极行内镜活检,尤其是十二指肠部位的活检,以明确诊断。

Objective To identify the clinical features of adult patients with autoimmune enteropathy(AIE)in Chinese.Methods A respective study was conducted with the clinical data of 5 patients with adult AIE in Peking Union Medical College Hospital from 2011 to 2018,including clinical,endoscopic,histologic manifestations,medications and prognosis.Results Among the five patients,four were females,one was male,and their average age was 42 years old.The median disease duration was 2 months.The median hospitalization duration was 56 days.All patient initially presented with refractory diarrhea,electrolyte disturbance,severe malnutrition,and slight elevation of inflammatory markers.2 patients had hypovolemic shock and another 2 patients had Wernicke encephalopathy.Abdominal computed tomography findings including bowel-wall thickening,abnormal hyperenhancement of mucosal swelling,luminal dilatation,prominent mesenteric and retroperitoneal lymph nodes were nonspecific for AIE.Endoscopic appearances were mildly abnormal,including mucosal swellings and focal erosions.Specific histological features included villous blunting,absence of goblet or Paneth cells from the epithelium,increased crypt apoptotic bodies,lamina propria mononuclear infiltration,and surface intraepithelial lymphocytosis.Conclusions Clinical presentations and laboratory abnormalities of adult AIE are non-specific with minor endoscopic lesions.Therefore,endoscopic biopsy,especially duodenal biopsy is important and indispensable in patients with suspected adult AIE.