Central Nervous System Vasculitis in Systemic Lupus Erythematosus

Abstract

Necrotizing central nervous system (CNS) vasculitis in systemic lupus erythematosus (SLE) is rare, and its prognosis can be ominous. We describe a 49-year-old male with active SLE developing progressively deteriorating CNS symptoms due to CNS vasculitis. CT and MRI of the brain suggested vasculitis, and this was confirmed by brain biopsy. The patient was successfully treated with oral cyclophosphamide (2 mg/kg/day) and prednisone (1 mg/kg/day) following a 5-day course of intravenous (IV) pulse methylprednisolone (1 g/day). The diagnosis of CNS vasculitis should be considered in an SLE patient with deteriorating CNS symptoms and investigated first with CT and/or MRI; in suspected cases, brain biopsy should be performed. Once the diagnosis is established, early institution of daily oral cyclophosphamide and high-dose prednisone following a short course of pulse dose IV methylprednisolone can be used for its treatment and can result in improvement in outcome.