Chronic active Epstein–Barr virus infection manifesting as coronary artery aneurysm and uveitis

Abstract

Background Chronic active Epstein–Barr virus (CAEBV) infection is a type of lymphoproliferative disorder characterized by chronic or recurrent infectious mononucleosis (IM)-like symptoms, which can have less-frequent clinical presentations. The prognosis of CAEBV is poor, and hematopoietic stem cell transplantation (HSCT) has been shown to be the only potentially effective treatment. In this article, we present a special CAEBV case of a patient who had no typical IM-like symptoms at the early stage, but manifested with severe and progressive coronary artery aneurysm (CAA), abdominal aortic lesions, and severe uveitis. These manifestations were uncommon features and could only be blocked by HSCT. Case presentation A 4-year-old girl with no special medical history complained of decreased vision for 10 months and cough after physical activities for three months. The blurred vision grew rapidly worse within one month, until only light perception remained. She was diagnosed with uveitis and cataract, and received prednisone and ciclosporin A treatment. However, her vision did not improve. Physical examination showed slight hepatosplenomegaly. Ultrasonic cardiogram showed bilateral CAA (5.0 mm and 5.7 mm for inner diameters), and abdominal CT scan revealed a thickened aortic wall, as well as stenosis and dilation of the segmental abdominal aorta. Other significant findings were increased EBV-DNA (3.29 × 104 copies/mL) from peripheral blood, positive EBV antibodies (EBV-CA-IgG, EBV-EA-IgA, and EBV-NA-IgG), and positive EBV-encoded small RNAs found by bone marrow biopsy. Based on her clinical manifestations and evidence for EBV infection, we diagnosed CAEBV. She received allogeneic HSCT, and the cataract operation was performed after HSCT. EBV-DNA could not be detected in peripheral blood after HSCT. Her CAAs did not progress, and uveitis was well controlled. Her vision recovered gradually over the 3 years after HSCT. Conclusions We present a rare CAEBV case of a patient who suffered from uncommon and severe cardiovascular and ocular involvement that was relieved by HSCT. Therefore, early recognition and diagnosis of CAEBV are of vital importance to improve its prognosis. In summary, this atypical CAEBV case could help us recognize similar cases more easily, make the right diagnosis as early as possible, and deliver proper and timely treatment.

Case Report: Pediatric Chronic Active Epstein-Barr Virus Infection With Giant Sinus of Valsalva Aneurysms and Aorta and Its Branch Dilations

“…In 1988, Kobayashi et al first reported one child with CAEBV accompanied by dilation of left and right coronary arteries (14). Up to now, all cases of patients with CAEBV associated with CAL are reported from Japan (4,(6)(7)(8)15) and China (12,(16)(17)(18). Our patient in this case had multiple severe cardiovascular complications, her ECG showed second degree of AV block, and the echocardiogram revealed moderate mitral valve and tricuspid regurgitation, mild aortic regurgitation, bilateral SoV aneurysms, bilateral coronary artery lesions, aorta, and its branch dilations.…”

Epstein-Barr virus infection associated polymyositis and coronary artery dilation

“…The elevated myocardial viral load, positive EBV protein in cardiomyocytes, and inflamed coronary intramural vessels confirmed the causal role of EBV [ 14 ]. Cardiovascular lesions in CAEBV could be strongly associated with EBV-infected T or NK lymphocyte infiltration and injuries in the vessel walls, and EBV-induced high levels of inflammatory cytokines [ 5 ]. Our case developed CAD when her clinical symptoms improved.…”

Henoch-Schönlein Purpura /IgA Vasculitis Complicated by Coronary Artery Aneurysm: A Case Report and Literature Review

“…In recent years, other rheumatic diseases such as juvenile idiopathic arthritis (sJIA) and systemic lupus erythematosus (SLE) have also been reported to be complicated with coronary artery dilatation (34,35). In addition, there are some reports of coronary artery dilatation in patients with infectious diseases such as EBV virus infection and COVID-19 (36,37). It indicates that coronary artery dilatation may be more common than previously recognized.…”