Vascular Complications in Chronic Active Epstein–Barr Virus Disease: Clinical Spectrum, Pathogenic Mechanisms, and Potential Disease Modifiers
- 1Department of Anesthesiology and Pain Medicine, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, Republic of Korea
- 2Department of Genetics, Yale Stem Cell Center, Yale School of Medicine, New Haven, CT 06520, USA
Abstract
Chronic active Epstein–Barr virus disease (CAEBV) is a rare, potentially life-threatening EBV-positive T/NK-cell lymphoproliferative disorder characterized by persistent systemic inflammation and multiorgan involvement. Although hemophagocytic lymphohistiocytosis and progression to lymphoid malignancy are well-recognized complications, vascular manifestations—including vasculitis, arterial dilatation and aneurysm formation, intracranial hemorrhage, and vascular involvement of multiple organ systems—remain poorly characterized. Available evidence suggests that persistent activation of EBV-infected T/NK cells, cytokine-mediated hyperinflammation, endothelial injury, and coagulation abnormalities may contribute to CAEBV-associated vasculopathy, whereas the roles of complement activation and thrombotic microangiopathy remain less well established. This review synthesizes the clinical spectrum and proposed mechanisms of vascular complications in CAEBV, with particular attention to the strength of evidence supporting individual pathogenic pathways. Improved recognition of vascular manifestations, application of contemporary CAEBV diagnostic criteria, and further mechanistic and multicenter studies are needed to improve risk stratification and management.
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BibTeX
@article{kim2026vascular,
title = {{Vascular Complications in Chronic Active Epstein–Barr Virus Disease: Clinical Spectrum, Pathogenic Mechanisms, and Potential Disease Modifiers}},
author = {Jong-Woan Kim and Woo Sub Yang and Minji Park and Hyun Jung Koh},
journal = {International Journal of Molecular Sciences},
year = {2026},
month = oct,
doi = {10.3390/ijms27219404},
url = {https://www.mdpi.com/1422-0067/27/21/9404},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Vascular Complications in Chronic Active Epstein–Barr Virus Disease: Clinical Spectrum, Pathogenic Mechanisms, and Potential Disease Modifiers
AU - Jong-Woan Kim
AU - Woo Sub Yang
AU - Minji Park
AU - Hyun Jung Koh
PY - 2026
DA - 2026/10/22
JO - International Journal of Molecular Sciences
DO - 10.3390/ijms27219404
UR - https://www.mdpi.com/1422-0067/27/21/9404
AB - Chronic active Epstein–Barr virus disease (CAEBV) is a rare, potentially life-threatening EBV-positive T/NK-cell lymphoproliferative disorder characterized by persistent systemic inflammation and multiorgan involvement. Although hemophagocytic lymphohistiocytosis and progression to lymphoid malignancy are well-recognized complications, vascular manifestations—including vasculitis, arterial dilatation and aneurysm formation, intracranial hemorrhage, and vascular involvement of multiple organ systems—remain poorly characterized. Available evidence suggests that persistent activation of EBV-infected T/NK cells, cytokine-mediated hyperinflammation, endothelial injury, and coagulation abnormalities may contribute to CAEBV-associated vasculopathy, whereas the roles of complement activation and thrombotic microangiopathy remain less well established. This review synthesizes the clinical spectrum and proposed mechanisms of vascular complications in CAEBV, with particular attention to the strength of evidence supporting individual pathogenic pathways. Improved recognition of vascular manifestations, application of contemporary CAEBV diagnostic criteria, and further mechanistic and multicenter studies are needed to improve risk stratification and management.
SN - 1422-0067
ER -