Neuropsychiatric manifestations in childhood-onset systemic lupus erythematosus

Neuropsychiatric manifestations occur frequently and are challenging to diagnose in childhood-onset systemic lupus erythematosus (SLE). Most patients with childhood-onset SLE have neuropsychiatric events in the first 2 years of disease. 30-70% of patients present with more than one neuropsychiatric event during their disease course, with an average of 2-3 events per person. These symptoms are associated with disability and mortality. Serum, cerebrospinal fluid, and neuroimaging findings have been described in childhood-onset SLE; however, only a few have been validated as biomarkers for diagnosis, monitoring response to treatment, or prognosis. The aim of this Review is to describe the genetic risk, clinical and neuroimaging characteristics, and current treatment strategies of neuropsychiatric manifestations in childhood-onset SLE.