Clinical characteristics of 10 patients of chronic active Epstein-Barr virus infection with intestinal involvement misdiagnosed as inflammatory bowel disease
Objective To analyze the clinical characteristics of patients of chronic active Epstein-Barr virus infection(CAEBV)with intestinal involvement misdiagnosed as inflammatory bowel disease(IBD).Methods A retrospective analysis was conducted on the clinical characteristics,laboratory results,digestive endoscopic findings,histological results,treatment and prognosis of 10 patients with CAEBV intestinal involvement who were misdiagnosed as IBD and treated at the Department of Hematology,Beijing Friendship Hospital,Capital Medical University from February 2019 to November 2022.Epstein-Barr virus-encoded small RNA(EBER)was detected by in situ hybridization.Results Among the 10 patients with CAEBV,eight were males and two were females.Seven patients had been misdiagnosed as ulcerative colitis and three misdiagnosed as Crohn's disease.The median age of onset was 36 years(ranged from 26 to 52 years),and the median time from onset to CAEBV diagnosis was 18.5 months(ranged from 2.0 to 96.0 months).The main clinical characteristics of these patients included fever＞38.5℃in 10 cases,diarrhea in seven cases,abdominal pain in seven cases,abdominal lymph node enlargement in six cases and hematochezia in seven cases.Six patients primarily presented with gastrointestinal symptoms,and seven patients had involvement of extraintestinal organs,three patients developed hemorrhagic shock due to gastrointestinal bleeding.The laboratory findings included anemia in seven cases,elevated erythrocyte sedimentation rate in six cases,decreased natural killer cell activity in five cases,and elevated ferritin in three cases.Epstein-Barr virus(EBV)DNA were detected in the peripheral blood mononuclear cells(PBMCs)of nine patients,with a median viral load of 23 000 copies/mL.Seven patients were tested positive for anti-EBV viral capsid antigen IgG and nuclear antigen 1 IgG.The main endoscopy findings were hyperemia,edema of the affected intestinal wall mucosa,which could be accompanied by erosion,multiple scattered shallow ulcers with varying sizes.There were six patients with total colon involvement.The rectum was involved in three patients,and the esophagus,gastric antrum,duodenum and small intestine were each involved in one patient.Seven patients underwent follow-up colonoscopy after diagnosis,and four cases progressed.All 10 patients showed active chronic inflammation in the histopathological examinations of their intestinal tissue,with crypt changes in four cases and granulomatous changes in one cases.The intestinal tissues of eight patients were positive for EBER staining,and EBER positive cells ≥ 50 cells/high-power field in seven patients.Seven patients were treated with 5-aminosalicylic acid before the correct diagnosis.Five patients had not improved or progressed upon the follow-up colonoscopy.Two patients died of uncontrolled massive hemorrhage of digestive tract.Conclusions The clinical,endoscopic and pathological findings of patients with CAEBV intestinal involvement lack specificity.For IBD patients initially diagnosed accompanied by fever and evidence of extraintestinal organ involvement,it is recommended to simultaneously detect EBV DNA in PBMCs and blood plasma,EBER in intestinal tissue,and identify the main EBV-infected cells in peripheral blood and/or tissue,to distinguish CAEBV.
IntestinesInflammatory bowel diseasesChronic active Epstein-Barr virus infectionClinical characteristics
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