Vascular Complications in Chronic Active Epstein–Barr Virus Disease: Clinical Spectrum, Pathogenic Mechanisms, and Potential Disease Modifiers

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.