Cardiovascular Complications Associated with Chronic Active Epstein–Barr Virus Infection

Muneuchi, Jun; Ohga, Shouichi; Ishimura, Masataka; Ikeda, Kazuyuki; Yamaguchi, Kenichiro; Nomura, Akihiko; Takada, Hidetoshi; Abe, Yasunobu; Hara, Toshiro

doi:10.1007/s00246-008-9343-8

Cited by 31 publications

(37 citation statements)

References 32 publications

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“…Kikuta et al (11,12) reported that the EBV DNA sequence was detected in 83% of KD patients and in 18% of control subjects. Chronic active EBV infection sometimes involves CALs, including coronary artery aneurysms (13,14). Although the pathogenesis of EBVinfection-associated CALs is unclear, it has been demonstrated in vitro that deoxyuridine 5'-triphosphate nucleotidohydrolase (dUTPase), an EBV-encoded protein, stimulates monocytederived macrophages through Toll-like receptor 2 (TLR2)dependent signaling (15).…”

Section: Epstein-barr Virusmentioning

confidence: 99%

Aetiological Significance of Infectious Stimuli in Kawasaki Disease

2019

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Kawasaki disease (KD) is a pediatric vasculitis syndrome that is often involves coronary artery lesions (e. g., coronary artery aneurysms). Although its causal factors and entire pathogenesis remain elusive, the available evidence indicates that the pathogenesis of KD is closely associated with dysregulation of immune responses to various viruses or microbes. In this short review, we address several essential aspects of the etiology of KD with respect to the immune response to infectious stimuli: 1) the role of viral infections, 2) the role of bacterial infections and the superantigen hypothesis, 3) involvement of innate immune response including pathogens/microbe-associated molecular patterns and complement pathways, and 4) the influence of genetic background on the response to infectious stimuli. Based on the clinical and experimental evidence, we discuss the possibility that a wide range of microbes and viruses could cause KD through common and distinct immune processes.

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Section: Epstein-barr Virusmentioning

confidence: 99%

Aetiological Significance of Infectious Stimuli in Kawasaki Disease

2019

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“…However, in some cases of EBV infection a chronic active viral infection of T lymphocytes and natural killer cells may be detected. Previous studies have described CAEBV patients with cardiac complications, including coronary artery aneurism [7]. Thus, not only HLH but also CAEBV can cause severe cardiac symptoms similar to Kawasaki disease.…”

Section: Introductionmentioning

confidence: 99%

A case of Epstein-Barr virus–associated hemophagocytic lymphohistiocytosis with severe cardiac complications

et al. 2016

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BackgroundHemophagocytic lymphohistiocytosis (HLH) is a life threatening hematological disorder associated with severe systemic inflammation caused by an uncontrolled and ineffective immune response resulting in cytokine storm. Epstein-Barr virus (EBV) is the most common infectious agent in patients with the viral-associated HLH. Limited numbers of cases with cardiac complication have been demonstrated in other viral-associated HLH patients. Herein, we report a pediatric case of severe EBV-associated HLH with cardiac complications.Case presentationA previously healthy 4-year-old Japanese female was admitted to a local hospital with a four day history of fever. Despite antibiotic treatment, her fever persisted to day 7 of the illness. Finally, the diagnosis of HLH was confirmed by fulfilling diagnostic criteria for HLH and pathological analysis of bone marrow aspiration. Real-time PCR detected a high copy number of EBV DNA in the peripheral blood mononuclear cells (PBMCs) at the time of hospital admission. During treatment according to HLH-2004 protocol, sudden cardiopulmonary arrest (CPA) occurred on day 30 of the illness and immediate resuscitation was successful. Acute myocarditis was considered the cause of the CPA. Although the treatment regimen was completed on day 88 of the illness, a remarkably high copy number of EBV DNA was still detected in her PBMCs. Based on our flow cytometric in situ hybridization analysis that revealed EBV infection of only B lymphocytes, we decided to administer rituximab to control the abnormal EBV infection. Afterwards the amount of EBV DNA decreased gradually to undetectable level on day 130 of the illness. Unfortunately, a coronary artery aneurysm was discovered at the left main coronary artery on day 180 of the illness. Finally, the patient was discharged from the hospital on day 203 of the illness without sequelae except for a coronary aneurysm.ConclusionsIn this case report, EBV-HLH was complicated with cardiac symptoms such as myocarditis and coronary artery aneurysm. Although remarkably high copy number of EBV DNA was detected in PBMCs after completion of the HLH-2004 protocol, rituximab treatment resulted in a dramatic decrease of EBV DNA to undetectable levels. Rituximab treatment might have been beneficial for the patient’s survival.

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“…It has 2 forms: EBV-associated T/natural killer (NK) cell proliferation, and EBV-associated B cell proliferation 3 . In addition to chronic mononucleosis symptoms, the clinical spectrum includes mucocutaneous disease, uveitis, encephalitis, vasculitis, myocarditis, and hemophagocytosis, thus potentially mimicking rheumatic disease 1,2,3,4,5 .…”

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confidence: 99%

“…CAEBV has multiple manifestations that overlap with www.jrheum.org Downloaded on September 3, 2020 from autoimmune and inflammatory conditions, including vasculitis 4,5 . Rheumatologists should consider this rare diagnosis when evaluating patients with complex clinical presentations.…”

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confidence: 99%