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Published on: September 7, 2022
Chronic active Epstein-Barr virus infection involving gastrointestinal tract mimicking inflammatory bowel disease
Weijia Xu1, Xiaoyun Jiang1, Jiajie Chen1
1Department of Gastroenterology, Huashan Hospital (North), Fudan University, Shanghai, China.
Insights
Chronic active Epstein-Barr virus infection (CAEBV) presents with fever and elevated ferritin, mimicking inflammatory bowel disease (IBD). Early detection of EBV-DNA and EBER is crucial for differentiating CAEBV, which has a poor prognosis.
Area of Science:
- Infectious Diseases
- Gastroenterology
- Oncology
Background:
- Chronic active Epstein-Barr virus infection (CAEBV) is a rare condition that can be mistaken for inflammatory bowel disease (IBD).
- This study focuses on immunocompetent patients with gastrointestinal involvement from CAEBV.
- Twelve CAEBV cases are presented to raise awareness and aid differential diagnosis.
Purpose of the Study:
- To highlight the clinical, endoscopic, and prognostic features of CAEBV in immunocompetent patients.
- To differentiate CAEBV from IBD based on specific clinical and laboratory findings.
- To emphasize the importance of early diagnostic methods for CAEBV.
Main Methods:
- Retrospective analysis of twelve CAEBV patients meeting diagnostic criteria.
- Comparison with a control group of twenty-four IBD patients with elevated EBV-DNA.
- Review of clinicopathologic and endoscopic characteristics.
Main Results:
- CAEBV patients commonly presented with intermittent fever (100%), hepatosplenomegaly (58%), lymphadenopathy (50%), diarrhea (50%), and hematochezia (50%).
- Intermittent fever and elevated ferritin levels were significantly higher in CAEBV patients compared to IBD patients.
- Endoscopic findings in CAEBV included irregular ulcers and diffuse inflammation, lacking the typical cobblestone appearance seen in IBD. Median EBV-DNA levels were significantly higher in CAEBV patients.
- Ten CAEBV patients died within 5 years, with an average survival of 21 months.
Conclusions:
- Intermittent fever, elevated ferritin, and atypical endoscopic findings suggest CAEBV.
- Early detection of EBV-DNA in serum and EBV-encoded small nuclear RNA (EBER) via in situ hybridization in intestinal tissue are critical for distinguishing CAEBV from IBD.
- Prompt diagnosis is essential due to the severe prognosis of CAEBV.
Background:
Chronic active Epstein-Barr virus infection (CAEBV) is a rare disease, which is difficult to be differentiated from inflammatory bowel disease (IBD). To cause the attention, we present twelve cases of CAEBV in immunocompetent patients with gastrointestinal tract involvement.
Methods:
Twelve patients who fulfilled the diagnostic criteria of CAEBV were enrolled in this retrospective study. The control group was consisted of twenty-four IBD patients with EBV-DNA value increased in peripheral blood. The clinicopathologic and endoscopic characteristics were reviewed and analyzed.
Results:
The major clinical presentations of CAEBV patients were intermittent fever (100%), hepatomegaly/splenomegaly (58%), lymphadenopathy (50%), diarrhea (50%) and hematochezia (50%). Compared with IBD patients, the incidence of intermittent fever and increased level of ferritin were significantly higher among CAEBV patients. The median values for EBV detected in peripheral blood were significantly higher in CAEBV group (1.42*10^6 copies/μg) than in IBD group (3.2*10^3 copies/μg, p<0.05). The main endoscopic findings of CAEBV included multifocal or isolated, irregular, multiform ulcers and diffuse inflammation, lacking of typical cobblestone appearance. Ten patients died within 5 years of disease onset. The average survival time is 21 months.
Conclusions:
Symptoms such as intermittent fever, increased level of ferritin and atypical endoscopic findings could be a sign for CAEBV. Early detections of EBV-DNA in serum and EBV-encoded small nuclear RNA (EBER) by in situ hybridization in intestinal tissue are essential for differential diagnosis between CAEBV and IBD.
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