Chronic active Epstein-Barr virus infective enteritis (CAEBV enteritis) is rare and has not been well described yet. Therefore, we reported the clinicopathologic features of 11 patients with chronic active Epstein-Barr virus infective enteritis and their differences from inflammatory bowel disease. The major clinical presentations of chronic active Epstein-Barr virus infective enteritis were intermittent fever over 39 degrees C (100%), diarrhea (73%), abdominal pain (64%), lymphadenopathy (64%), splenomegaly (64%), and hepatomegaly (27%). The main endoscopic findings included numerous shallow, small, and irregular ulcers, mainly involving colon and small intestine together, no cobble-like appearance, and longitudinal ulcers. Compared to inflammatory bowel disease patients, the frequency of intermittent fever, hepatomegaly, splenomegaly, lymphadenopathy, the value of C-reactive protein, and serum Epstein-Barr virus DNA (EBV DNA) were significantly higher in chronic active Epstein-Barr virus infective enteritis patients (p < 0.01). The histologic findings show transmural inflammation with extended lymphoid infiltration, fissuring ulcers, and intraepithelial lymphocytosis. But chronic active Epstein-Barr virus infective enteritis lacked granulomas and connective tissue changes such as neural hypertrophy and thickened muscularis mucosae. Three chronic active Epstein-Barr virus infective enteritis patients died within 5 years of disease onset, and those three patients have received total colectomy, two of them died within 1 month after surgery. In this study, clinicopathologic features have been summarized to better recognize chronic active Epstein-Barr virus infective enteritis. There are resemblances between chronic active Epstein-Barr virus infective enteritis and inflammatory bowel disease, but some symptoms, signs, and indexes like intermittent fever, hepatomegaly, splenomegaly, lymphadenopathy, and elevated C-reactive protein, Epstein-Barr virus DNA are characteristics to differentiate chronic active Epstein-Barr virus infective enteritis and inflammation. Histopathological features also help the discrimination. Serum Epstein-Barr virus DNA and intestinal tissue in situ hybridization for Epstein-Barr virus-encoded RNA are recommended to exclude chronic active Epstein-Barr virus infective enteritis.
[1]Zhejiang Univ, Dept Gastroenterol, Sir Run Run Shaw Hosp, Sch Med, Hangzhou, Zhejiang, Peoples R China;
[2]Zhejiang Univ, Ctr Inflammatory Bowel Dis, Sir Run Run Shaw Hosp, Sch Med, Hangzhou, Zhejiang, Peoples R China;
[3]Mayo Clin, Dept Lab Med & Pathol, Rochester, NY USA;
[4]Chinese Acad Med Sci, Peking Union Med Coll Hosp, Dept Pathol, Beijing, Peoples R China;
[5]Fudan Univ, Childrens Hosp, Shanghai, Peoples R China;
[6]Third Mil Med Univ, Xinqiao Hosp, Dept Gastroenterol, Chongqing, Peoples R China;
[7]Shanghai Jiao Tong Univ, Sch Med, Rui Jin Hosp, Dept Gastroenterol, Shanghai, Peoples R China;
[8]Zhejiang Univ, Ctr Inflammatory Bowel Dis, Dept Gastroenterol, Affiliated Hosp 2,Sch Med, Hangzhou, Zhejiang, Peoples R China;
[9]Jilin Univ, Bethune Affiliated Hosp 1, Dept Gastroenterol, Changchun, Jilin, Peoples R China;
[10]Zhejiang Univ, Sch Med, Affiliated Hosp 1, Hangzhou, Zhejiang, Peoples R China;
[11]Sichuan Univ, West China Hosp, Dept Gastroenterol, Chengdu, Sichuan, Peoples R China;
[12]Fudan Univ, Huashan Hosp, Shanghai, Peoples R China;
[13]Peking Union Med Coll Hosp, Dept Gastroenterol, Beijing, Peoples R China;
[14]Zhejiang Univ, Dept Pathol, Sir Run Run Shaw Hosp, Sch Med, Hangzhou, Zhejiang, Peoples R China
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