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Lupus, Vol. 16, No. 8, 564-571 (2007)
DOI: 10.1177/0961203307078971

Reviews

Review: Neuropsychiatric involvement in pediatric systemic lupus erythematosus

S.M. Benseler

Divisions of Rheumatology, Department of Paediatrics and Immunology, The Hospital for Sick Children, University of Toronto, Toronto, Canada

E.D. Silverman

Divisions of Rheumatology, Department of Paediatrics and Immunology, The Hospital for Sick Children, University of Toronto, Toronto, Canada, Earl.Silverman{at}sickkids.ca

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. Lupus (2007) 16, 564—571.

Key Words: anti-phospholipid antibodies • central nervous system • children • lupus • outcome


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