Results
Together with our own case report, a total of 27 case reports[7-13] described CAEBV. A summary is shown in Table 2 and Table 3.
  1. Basic information: The age of onset was mostly concentrated in 20-40 year olds and 40-60 year olds. The average patient age was 40 years. The ratio of males to females was 4:1 (21 M:6 F). The disease course was less than half a year (n = 6) and more than one year (n = 7). The median disease course was 29.4 months (range 0.5-14 years).
  2. Clinical manifestations: Fever (25/27), abdominal pain (14/27), diarrhoea (16/27), and bloody stool (13/27) were the most common symptoms and the first symptoms. Combined with lymphadenopathy and hepatosplenomegaly. High fever (body temperature > 39 ℃), intestinal bleeding, intestinal perforation and septic shock occurred in the late stage of the disease. Other symptoms were not typical, such as loss of appetite, weight loss, nausea and vomiting. There were no obvious extraintestinal manifestations, such as skin damage, joint swelling and pain, or ophthalmitis. All patients were in good health and had no history of immune deficiency, tumours, family history or previous immunosuppressive drug treatment.
  3. Laboratory examinations: In routine blood tests, decreases in haemoglobin and red blood cell counts (14/27) were common, as well as decreases in platelet and white blood cell (WBC) counts. Increases in WBC counts and C-reactive protein (CRP) were common. Coagulation function was abnormal. Immunological tests, such as TB-Ab, T-SPOT, hepatitis A and hepatitis C, were negative. All 27 cases were confirmed by histopathology, and EBER was positive based on in situ hybridization. The average DNA load of EBV in the serum was 6.3*10^5 copies/ml.
  4. Endoscopic features: All patients had colonic and rectal ulcers of varying degrees, with ulcers that were deep, shallow, and of varying sizes accompanied by luminal narrowing and no apparent specificity for ulceration. The small and large intestines were mostly involved. CAEAE was often misdiagnosed as Crohn’s disease (CD), ulcerative colitis (UC), intestinal tuberculosis, etc. Pathological characteristics: Most patients had full-thickness chronic and acute Intestinal mucosal inflammation with the infiltration of lymphocytes and plasma cells without obvious structural changes in crypts and occasionally cryptitis and crypt abscesses. The pathological findings were not typical, and there were no definite pathological features, such as lymphoma, CD, and UC.
  5. Nine patients were treated surgically, mostly with emergency surgery, and seven died postoperatively due to infection, rebleeding, perforation, and shock. The mortality rate was 77.7%, and three patients (3/7) died within 1 month of surgery. Twelve patients did not undergo surgery and were medically treated, four of whom died, seven of whom remained alive, and one of whom was lost to follow-up. The mortality rate was 33.3%. There were significant differences in surgical and nonsurgical survival (P < 0.05). The proportion of deaths within 6 months was as high as 45.4%.
  6. Twenty-one individuals were treated with medications, mainly glucocorticoids (prednisone, methylprednisolone), antiviral agents (ganciclovir), immunoglobulins for injection, immunosuppressive agents (methotrexate, azathioprine), antibiotics (cefoperazone sodium, sulbactam sodium, and vancomycin), mesalazine, infliximab, Chinese medicine, etc.