Objective: To investigate the clinical and pathological characteristics of chronic active Epstein-Barr virus associated enteritis (CAEAE) . Methods: The clinical data of 6 CAEAE patients in Peking Union Medical College Hospital were retrospectively analyzed from January 2010 to November 2017, including clinical manifestations, endoscopic, pathological features, medications and clinical outcome. Results: The male∶female ratio was 4∶2 and the average age was 34 years old. All patients did not have personal and family history of immunodeficiency. The common symptoms consisted of fever (6/6), abdominal pain (6/6), hematochezia (6/6) and diarrhea (5/6). The most frequently affected sites were colorectum (5/6), followed by small intestine (3/6). Further serologic tests revealed a high load of serum Epstein-Barr virus(EBV) DNA. The main manifestations under endoscopy were multifocal or diffuse irregular ulcers. There was inflammation in the ulcer bases and surrounding areas, where EBV-encoded small RNA (EBER) was positive by in situ hybridization (6/6). The common complications were massive hemorrhage (3/6) and intestinal perforation (2/6). None of treatment agents including glucocorticoid, ganciclovir, foscarnet sodium and cytotoxic drugs was effective. All patients died within 0.5 to 13 months after diagnosis. Conclusions: CAEAE in immunocompetent individuals is a rare disorder with poor prognosis. It is difficult to differentiate CAEAE from inflammatory bowel disease due to similar clinical and endoscopic manifestations.
目的： 通过分析非免疫缺陷个体中慢性活动性EB病毒感染相关性肠炎（CAEAE）的临床、内镜和病理特点，提高对其的认识及早期诊断水平。 方法： 回顾性分析北京协和医院2010年1月1日至2017年11月1日收治的6例CAEAE患者的临床表现、内镜检查结果及治疗随访情况。 结果： 6例CAEAE患者中，男4例，女2例，平均年龄34岁，均无免疫缺陷病史及免疫抑制药物治疗史。主要临床表现包括高热（6/6）、腹痛（6/6）、便血（6/6）、腹泻（5/6），病程中常出现急性消化道大出血（5/6）。全部患者外周血EBV DNA水平明显升高。内镜下主要表现为肠道多发溃疡（6/6），形态呈多样性，最常累及结肠（5/6），其次为小肠（3/6）、上消化道（2/6）。病变处肠黏膜行原位杂交检测EB病毒编码小RNA（EBER）为阳性（6/6）。常出现严重并发症如失血性休克（3/6）、肠穿孔（2/6），急诊手术率为3/6。全部患者接受过糖皮质激素治疗，其他药物包括更昔洛韦、膦甲酸钠及细胞毒性药物，效果不明显。全部患者在诊断后0.5~13个月内死亡。 结论： 非免疫缺陷个体中出现的CAEAE临床罕见，表现危重，预后不佳；症状及内镜下表现缺乏特异性，易被误诊为炎症性肠病。临床医师应提高认识，对于出现高热、便血、结肠溃疡而病因不明的患者行EB病毒血清学及组织学检测有助于提高确诊率。.
Keywords: Clinical features; Enteritis; Epstein-Barr virus infection.