Abstract

Aim To improve the identification and computed tomography (CT) diagnostic accuracy of chronic active Epstein-Barr virus (EBV)-associated enteritis (CAEAE) by evaluating its CT findings and clinical manifestation. Methods The data of three patients with pathologically and clinically confirmed CAEAE who underwent CT enterography (CTE) were retrospectively reviewed from January 2018 to October 2019. The following data were evaluated: imaging characteristics (length of involvement, pattern of mural thickening, pattern of attenuation, perienteric abnormalities), clinical symptoms, endoscopic records, laboratory examinations, and pathologic findings. Results Based on CT findings, two patients demonstrated segmental bowel wall thickening (involvement length >6 cm), asymmetric thickening, layered attenuation, fat stranding, and adenopathy, whereas the remaining one had no positive finding. The endoscopic results of all patients showed numerous irregular ulcers in the colon, and one patient had a focal esophageal ulcer. The major clinical symptoms were abdominal pain (n = 3), retrosternal pain (n = 1), fever (n = 3), diarrhea (n = 2), hematochezia (n = 1), and adenopathy (n = 3). The main laboratory examination indicators were increased serum EBV DNA load (n = 1) and increased inflammatory markers (n = 3). With regard to the main pathologic findings, all patients showed positive EBV-encoded RNA (EBER) situ hybridization in the colonic biopsy specimen, with one patient being positive in the esophagus. Conclusion CAEAE is rare and is usually misdiagnosed as inflammatory bowel disease (IBD). The imaging features of CAEAE overlap with those of Crohn's disease and ulcerative colitis. The presence of segmental and asymmetric bowel wall thickening, layered attenuation, and fat stranding in the CTE image may be helpful in differentiating CAEAE from IBD.

Highlights

  • Chronic active Epstein-Barr virus-associated infection (CAEBV) is one of the many subtypes of Epstein-Barr virus (EBV)-associated lymphoproliferative disorders (EBV-LPD) and comprises a range of lymphoid tissue diseases including hyperplastic, borderline, and neoplastic diseases [1]

  • The diagnostic criteria for Chronic active EBV-associated enteritis (CAEAE) were based on a previous study [15]: (1) recurrent or persistent infectious mononucleosis-like symptom: (a) swelling of lymph nodes, fever, and hepatosplenomegaly; (b) additional complications including digestive tract, hematological, neurological, pulmonary, ocular, dermal, and/or cardiovascular disorders that mostly have been reported in patients with mononucleosis infection

  • (2) An unusual pattern of anti-EBV antibodies with elevated anti-EA and antiVCA and/or detection of increased EBV genomes in affected tissues, including peripheral blood

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Summary

Introduction

Chronic active Epstein-Barr virus-associated infection (CAEBV) is one of the many subtypes of Epstein-Barr virus (EBV)-associated lymphoproliferative disorders (EBV-LPD) and comprises a range of lymphoid tissue diseases including hyperplastic, borderline, and neoplastic diseases [1]. In a few individuals, infected lymphocytes including T, B, and natural killer (NK) cells selectively proliferate into cells with multiple mixed types of clonality, which cause EBV-LPD [4,5,6]. Chronic active EBV-associated enteritis (CAEAE) was first described in 2005 by Joan Robinson et al [10]. Some papers in the literature have reported the clinical and pathological characteristics of CAEAE; the detailed imaging characteristics of CAEAE have not been well described [11,12,13,14]. CAEAE is diagnosed mainly based on the clinical and pathological features of the patient, computed tomography (CT) enterography (CTE) imaging may provide a more evident information on changes

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