Abstract
Background: Chronic active Epstein-Barr virus (EBV)
infection-associated enteritis (CAEAE) in nonimmunodeficient individuals
is rare.
Methods: To report a case of CAEAE, relevant articles were
searched through databases. The clinical manifestations, endoscopic
findings, strategies of treatment, prognoses, and follow-up results of
CAEAE patients were analysed. Including this report, 7 citations in the
literature provide descriptions of 27 cases of CAEAE.
Results: There were 21 males and 6 females, with a mean age of
40 years. The main clinical manifestations were fever (25/27), abdominal
pain (14/27), diarrhoea (16/27), haematochezia or bloody stools (13/27),
and decreased haemoglobin and red blood cell counts in routine blood
tests (14/27). Elevations in inflammatory markers, white blood cell
(WBC) counts and C-reactive protein (CRP) were common. Coagulation was
often abnormal. Histopathology confirmed EBV-encoded small nuclear RNA
(EBER) in the affected tissue via in situ hybridization. The average
serum EBV DNA load was 6.3 x 10 ^ 5 copies/ml. All patients had
varying degrees of intestinal ulcers endoscopically, and the ulcers and
pathology were uncharacterized and misdiagnosed mostly as inflammatory
bowel disease (IBD). The course of the disease was progressive and later
complicated by intestinal bleeding, intestinal perforation, septic
shock, and a high rate of emergency surgery. However, the conditions of
the patients often did not improve after surgery, and some patients soon
died due to reperforation or massive haematochezia. Hormone and
antiviral treatment had no obvious effect. There was a significant
difference in surgical and nonsurgical survival (P < 0.05).
The proportion of patients who died within 6 months was as high as
63.6% (7/11).
Conclusions: CAEAE belongs to a group of rare, difficult
conditions, has an insidious clinical course, has a high case fatality
rate, and may later develop into EBV-positive lymphoproliferative
disorder (EBV-LPD), which in turn leads to carcinogenesis. Clinicians
should raise awareness that in patients with multiple ulcers in the
intestine of unknown aetiology, attention should be paid to EBV serology
and histology to make the diagnosis as early as possible.