Pathogenesis of neurocognitive and neuropsychiatric manifestations in childhood-onset lupus: an overview

This review explores current understanding of neuropsychiatric systemic lupus erythematosus (NPSLE) of childhood onset, in particular neurocognitive impairment. As yet, fewer studies have focused on childhood onset NPSLE compared to adult onset NPSLE and diagnosis still involves the 1999 American College of Rheumatology case definitions of neuropsychiatric syndromes, which were developed for adults. Although a validated core set of neuropsychometric tests exist for childhood onset NPSLE, these still have limitations and possible biomarkers and newer neuroimaging modalities remain mostly experimental. Important differences exist between childhood and adult onset SLE and specifically with NPSLE, outlined in this review. Normal adolescent brain development also involves significant differences from adults, particularly in executive function and social cognition. These issues may impact on the pathogenesis of NPSLE during this vulnerable period and also influence their management options.