Neuropsychiatric involvement in pediatric systemic lupus erythematosus
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Updated: Jul 12, 2026

The bm12 Inducible Model of Systemic Lupus Erythematosus (SLE) in C57BL/6 Mice
Published on: November 1, 2015
1Divisions of Rheumatology, Department of Paediatrics and Immunology, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
Insights
Neuropsychiatric (NP) symptoms affect about 25% of pediatric lupus patients, often within the first year of diagnosis. While outcomes are generally good, some children may experience lasting damage.
Area of Science:
- Pediatric Rheumatology
- Pediatric Neurology
- Immunology
Background:
- Neuropsychiatric (NP) manifestations affect approximately 25% of children and adolescents with pediatric Systemic Lupus Erythematosus (pSLE).
- NP involvement frequently occurs within the first year of diagnosis in pediatric lupus patients.
- Common NP presentations include headaches, psychosis, cognitive dysfunction, and cerebrovascular disease.
Purpose of the Study:
- To summarize the current understanding of neuropsychiatric manifestations in pediatric SLE.
- To highlight common clinical presentations and associated factors.
- To review current treatment approaches and outcomes in NP-pSLE.
Main Methods:
- Literature review of studies on neuropsychiatric manifestations in pediatric SLE.
- Analysis of reported clinical presentations, diagnostic considerations, and treatment strategies.
- Evaluation of patient outcomes, including survival rates and long-term sequelae.
Main Results:
- Headaches, psychosis, cognitive dysfunction, and cerebrovascular disease are the most frequent NP manifestations in pSLE.
- Anti-phospholipid antibodies are linked to specific NP conditions like chorea, cerebrovascular disease, and seizures.
- Standard treatment involves immunosuppressive combination therapy, though prospective studies are lacking.
Conclusions:
- Neuropsychiatric involvement is a significant concern in pediatric SLE, requiring multidisciplinary management.
- While overall survival is high, a notable percentage of children experience disease flares and permanent NP damage.
- Further research into novel auto-antibodies, imaging, and therapies is warranted for improved patient outcomes.
Abstract:
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.
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