Successful treatment with rituximab for central nervous system vasculitis caused by Epstein-Barr virus-associated lymphoproliferative disorder with immunoglobulin M gammopathy
Juhee Lee,
Han Sang Lee,
Kon Chu
Affiliations
Juhee Lee
Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
Han Sang Lee
Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
Kon Chu
Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
Monoclonal gammopathy of undetermined significance (MGUS) is associated with several autoimmune conditions, including central nervous system (CNS) vasculitis. Epstein-Barr virus (EBV) is a pathogen capable of triggering a systemic immune response and is involved in the occurrence of a wide range of B-cell lymphoproliferative disorders. In systemic autoimmune diseases, EBV infection is suspected to play a central role in pathogenesis. Here, we present a case that was thought to be a systemic autoimmune disease and CNS vasculitis that developed after EBV infection, demonstrating that rituximab is effective for the treatment.
