Enigmatic CNS Lupus Vasculitis: A Heuristic Approach

Abstract

Systemic lupus erythematosus is an autoimmune disease affecting all organs of the body. It has a higher incidence in women and can affect the central nervous system (CNS) in 10% to 20% of cases. Any structure in the CNS can be involved, resulting in a varied and often complex symptomatology. CNS lupus vasculitis is an even rarer manifestation characterized by small vessel inflammation that can result in vessel thrombosis and necrosis. We present the case of a 31-year-old woman with a history of systemic lupus erythematosus and Sjögren’s syndrome who was treated at our institution for an acute exacerbation of lupus complicated by CNS lupus vasculitis. This case was particularly challenging due to concurrent bacteremia and a travel history that broadened the differential diagnosis widely, thus requiring our input as infectious disease consultants. CNS lupus vasculitis is a diagnosis of exclusion that requires a high level of clinical suspicion to arrive at a prompt diagnosis paired with aggressive management to guarantee good outcomes. In this report, we share our stepwise approach to this challenging case in a didactic manner to benefit trainees and independent infectious diseases providers who may encounter similar clinical scenarios in their practice.