CAJ | 학술논문

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.
목적 탐토성인자신면역성장병적림상병이학특점、진단、감별진단.방법 수집북경대학제삼의원진치적성인자신면역성장병1례,수집환자상세적림상자료、실험실검사、병리검사자료,급환자적치료、수방정황,결합문헌진행분석.결과 환자위41세남성,완고성복사3개월여,음식조정급항염치료무효.대편경검가견지방구,대편소단Ⅲ염색양성.소장조영시소장연동감약,소장추벽감소.결장경미견이상.장경활검시소장융모소실,은와상피내조망소체화림파세포수량증다,표면상피내림파세포수량상대교소,고유막가견다량림파단핵세포、장세포급중성립세포침윤.결장가견은와염급은와농종,여개변여소장상동.면역형광시항배상세포항체IgG양성.결합병사、다충보조검사급병리조직학제외료기타인기복사적질병,최종진단위자신면역성장병.환자경격소치료후전유,차수방8개월、정용격소후5개월미복발.결론 성인자신면역성장병비상한견,정학진단대치료유중요지도의의.조직학형태수주의여궤양성결장염、유미사、림파세포성장염등감별.진단수결합병사、림상증상、내경하표현、혈청학검사급조직학종합분석.
Objective 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.