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A Case of Chronic Active Epstein-Barr Virus Infection with Coronary Aneurysm

Journal of the Korean Pediatric Society 2001;44(6):687-693.
Published online June 15, 2001.
A Case of Chronic Active Epstein-Barr Virus Infection with Coronary Aneurysm
Hee Jeong1, Bong Seong Kim1, Ok Ja Choi1, Han Wook Yoo1, So Duk Lim2, Soo-Jong Hong1
1Department of Pediatrics, Asan Medical Center, College of Medicine, Ulsan University, Seoul, Korea
2Department of Diagnostic Pathology, Asan Medical Center, College of Medicine, Ulsan University, Seoul, Korea
관상동맥류를 동반한 만성 활동성 Epstein-Barr Virus 감염증 1례
정희1, 김봉성1, 최옥자1, 유한욱1, 임소덕2, 홍수종1
1울산대학교 의과대학 서울중앙병원 소아과
2울산대학교 의과대학 서울중앙병원 진단병리과
Correspondence: 
Soo-Jong Hong, Email: sjhong@amc.seoul.kr
Abstract
Chronic active Epstein-Barr virus infection(CAEBV) is a nonfamilial syndrome that shows a specific immunodeficiency for the Epstein-Barr virus(EBV). CAEBV is characterized by fever, lymphadenopathy, splenomegaly, hepatitis, interstitial pneumonitis, interstitial nephritis, and uveitis. Cardiovascular complications are rare in EBV infection. Patients with CAEBV show characteristically high titers of anti-viral capsid antigen(VCA) IgG antibody and anti-early antigen(EA) antibody, as well as relatively low titer of anti-EB nuclear antigen(EBNA) antibody. We experienced a case of CAEBV with giant coronary aneurysms, who was a 6-year-old boy. He had 5 episodes of high fever and cervical lymphadenopathy and hepatosplenomegaly. The 6 mm sized bilateral coronary aneurysms were detected by echocardiography at second admission. IgG antibodies to EBV was positive, whereas negative for IgM antibody. Antibodies to EA and EBNA were also positive. The EBV was detected in lymph node tissue by in situ hybridization, and in the peripheral blood and bone marrow by the PCR. Treatment has been done with ganciclovir and interferon (IFN)-α for 5 weeks. The EBV-PCR of peripheral blood was converted to negative from 12th day of treatment. At present he has followed well for two years without fever and enlargements of lymphnode, except large coronary aneurysm.
Key Words: Chronic active Epstein-Barr virus infection, Coronary aneurysm, Epstein-Barr virus, Coronary aneurysm


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