The clinicopathologic features of chronic active Epstein-Barr virus infective enteritis

Abstract

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 °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.