摘要目的 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.

abstractsObjective 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.