Neuropsychiatric involvement in pediatric systemic lupus erythematosus.
Neuropsychiatric (NP) manifestations are found in approximately 25% of children and adolescents with pediatric SLE (pSLE). In 70% of those, NP involvement will occur within the first year from the time of diagnosis. Headaches (66%), psychosis (36%), cognitive dysfunction (27%) and cerebrovascular disease (24%) are the most common presentations. The support of a psychiatrist is often required. Anti-phospholipid antibodies are associated with distinct NP disease entities and may be implicated in the pathogenesis of several manifestations of NP-pSLE including chorea, cerebrovascular disease and seizures. The role of novel auto-antibodies and imaging modalities is currently explored. The treatment of NP-pSLE is not based on prospective studies; however, an immunosuppressive combination therapy consisting of high doses of prednisone and a second line agent such as cyclophosphamide or azathioprine is commonly suggested for children with NP-pSLE. The role of novel therapies is currently studied. The outcome of children with NP-pSLE is relatively good. The overall survival is 95-97%, 20% of children experience a disease flare during childhood and 25% have evidence of permanent neuropsychiatric damage.
Benseler
S M
SM
Divisions of Rheumatology, Department of Paediatrics and Immunology, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
Silverman
E D
ED
D002561:Cerebrovascular Disorders / Q000209:etiology; D002648:Child; D003072:Cognition Disorders / Q000209:etiology; D006261:Headache / Q000209:etiology; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis* / Q000523:psychology*; D020945:Lupus Vasculitis, Central Nervous System / Q000175:diagnosis* / Q000523:psychology*; D008279:Magnetic Resonance Imaging; D001523:Mental Disorders / Q000209:etiology; D010523:Peripheral Nervous System Diseases / Q000209:etiology; D012640:Seizures / Q000209:etiology