Neuropsychiatric Systemic Lupus Erythematosus in Children

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by the presence of antinuclear antibodies and other autoantibodies, as well as a clinical course that is characterized by flares and remissions. The clinical presentation is diverse, ranging from a mild disease characterized by rash and arthritis to a severe life-threatening disease involving multiple organs. Approximately 25% of children with SLE have neuropsychiatric manifestations of SLE, which are a major cause of morbidity and mortality. Neuropsychiatric symptoms may be the initial presentation of SLE in children. The mortality rate is relatively low, but morbidity may be significant and permanent damage can occur. This article discusses the importance, known pathophysiologic mechanisms, clinical approach, and evidence-based therapeutic options for the diagnosis and management of neuropsychiatric lupus erythematosus in children and adolescents.