Systemic granulomatous arteritis associated with Epstein-Barr virus infection

A 61-year-old woman initially presented with symptoms and findings reminisc ent of infectious mononucleosis, and her illness then took a rapidly fatal course. Autopsy revealed widespread granulomatous arteritis, with multinucl eated giant cells but without eosinophils and fibrinoid necrosis, affecting small arteries and arterioles and infiltration of haemophagocytic histiocy tes into many organs. In situ hybridization with Epstein-Barr virus (EBV)-s pecific oligonucleotide probes showed positive signals in the infiltrating immune cells and epithelial and endothelial cells of the affected organs. E BV-associated haemophagocytic syndrome (EBV-AHS) with systemic granulomatou s arteritis was diagnosed. From the immunophenotypes of the infiltrating im mune cells, a possible role of CD4+ T-cells in the pathogenesis of this hae mophagocytic syndrome and granulomatous vasculitis was suggested.