Fatal Systemic Vasculitis Associated with Chronic Active Epstein-Barr Virus Infection.

Chronic active Epstein-Barr virus infection is a rare disease with an often fatal outcome. Cardiovascular complications are associated with a poor prognosis. We herein describe the clinical course of an adult patient with Epstein-Barr virus-associated systemic vasculitis complicated by multi-systemic aneurysmal disease. The vascular imaging showed multiple aneurysms involving coronary arteries, abdominal arteries, cerebral arteries, and vertebral arteries. Immunophenotyping analysis of peripheral blood lymphocytes revealed the presence of an increased number of double negative T cells. The patient received multiple lines of immunosuppressive therapy with no response. Unfortunately, he succumbed to a cerebral aneurysm rupture.