Recurrent Neuropsychiatric Systemic Lupus Erythematosus Presenting with Primary Central Nervous System Lymphoma

We report the case of a systemic lupus erythematosus (SLE) patient who was diagnosed with lupus cerebritis, vasculitis, Epstein-Barr virus infection, and consequently, lymphoma. The patient presented to the emergency department with dysarthria, dysphagia, left-sided hemiparesis, and hypesthesia. Initial brain magnetic resonance imaging (MRI) revealed multiple high signal intensities in the cortex and subcortex. Intravenous methylprednisolone and immunoglobulin administration did not improve her symptoms. However, intravenous rituximab improved the parenchymal and angiographic abnormalities. One month later, she developed another acute left-sided hypesthesia. Brain MRI revealed multiple rim-enhancing lesions with perilesional edema, different from the previous lesion. Intravenous methylprednisolone and cyclophosphamide did not improve her symptoms, and she was finally diagnosed with primary central nervous system lymphoma after brain biopsy. Neurological symptoms improved after chemotherapy. Since the incidence of primary central nervous lymphoma is relatively high in SLE patients, careful diagnosis and accurate treatment are needed.