摘要目的 探讨成人自身免疫性肠病的临床病理学特点、诊断、鉴别诊断.方法 收集北京大学第三医院诊治的成人自身免疫性肠病1例,收集患者详细的临床资料、实验室检查、病理检查资料,及患者的治疗、随访情况,结合文献进行分析.结果 患者为41岁男性,顽固性腹泻3个月余,饮食调整及抗炎治疗无效.大便镜检可见脂肪球,大便苏丹Ⅲ染色阳性.小肠造影示小肠蠕动减弱,小肠皱襞减少.结肠镜未见异常.肠镜活检示小肠绒毛消失,隐窝上皮内凋亡小体和淋巴细胞数量增多,表面上皮内淋巴细胞数量相对较少,固有膜可见多量淋巴单核细胞、浆细胞及中性粒细胞浸润.结肠可见隐窝炎及隐窝脓肿,余改变与小肠相同.免疫荧光示抗杯状细胞抗体IgG阳性.结合病史、多种辅助检查及病理组织学除外了其他引起腹泻的疾病,最终诊断为自身免疫性肠病.患者经激素治疗后痊愈,且随访8个月、停用激素后5个月未复发.结论 成人自身免疫性肠病非常罕见,正确诊断对治疗有重要指导意义.组织学形态需注意与溃疡性结肠炎、乳糜泻、淋巴细胞性肠炎等鉴别.诊断需结合病史、临床症状、内镜下表现、血清学检查及组织学综合分析.

abstractsObjective To investigate the clinicopathologic features of adult-onset autoimmune enteropathy (AIE).Methods A case of adult-onset AIE was described along with a literature review.Results A 41-year-old male patient was admitted for intractable diarrhea for more than three months despite of any dietary restriction or anti-inflammatory therapy.Fat globule was observed by stool examination and Sudan Ⅲ staining of the stool was positive.Enteroclysis showed weak movement and few plica of small intestine,while colonoscopy appeared normal.Small bowel biopsies revealed villus atrophy and increased crypt apoptotic bodies and lymphocytic infiltration in deep crypt.Although without significant surface introepithelial lymphocytosis,there were a large number of monocytes,lymphocytes,plasmacytes and neutrophilic granulocytes infiltrating in the lamina propria.Morphologically,the colonic mucous was similar to the small intestine although cryptitis and crypt abscess were significant in the former.Serum IgG antigoblet cell antibody was demonstrated by indirect immunofluorescence.Other causes of diarrhea were excluded on the base of medical history,histopathology and other accessory examinations before the diagnosis of AIE was made.The patient had a complete remission after steroid treatment without recurrence for eight months during the follow-up even after steroid withdrawal for five months.Conclusions AIE is exceedingly rare and timely diagnosis is important for successful therapy.Histological differential diagnoses should include ulcerative colitis,celiac disease,lymphocytic colitis,etc.The final diagnosis should be based on histological examination combined with the patient history,clinical manifestation,endoscopy finding and serological testing.