Clinical Features and Outcome of Neuropsychiatric Manifestations in Juvenile Onset Systemic Lupus Erythematosus.

OBJECTIVES

To assess the clinical characteristics and outcomes of children with neuropsychiatric systemic lupus erythematosus (NPSLE).

METHODS

A retrospective analysis of demographic, clinical and laboratory data of Juvenile onset systemic lupus erythematosus (jSLE) patients managed at a tertiary rheumatology clinic over 30 y was performed. Results were expressed as proportions, mean with standard deviation and median. Comparative analyses and multivariate logistic regression were used to identify independent predictors of NPSLE.

RESULTS

Among 338 jSLE patients, 25.1% patients had NP manifestations. The mean age at presentation was 13.86 y. Seizures were the most common manifestation (62.3%), followed by psychosis (17.6%), mood disorders (14.1%), neuropathies (8.2%), chorea (4.7%) and transverse myelitis (3.5%). On univariate analysis, serositis, nephritis, constitutional symptoms, higher Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores and lower anti-phospholipid antibodies levels were associated with NPSLE; however, these associations were not significant on multivariate analysis adjusted for age and sex. Among 69 patients with follow-up, (median 55 mo), 76.8% had no recurrences. Recurrent NP manifestations occurred in 10.1%. Residual damage was noted in 4 patients (hearing loss, foot drop, and quadriparesis). Five patients died (2 due to status epilepticus, 1 due to infection and 2 due to non-neurological causes).

CONCLUSIONS

NP manifestations occur in a quarter of patients with jSLE. Recurrences and significant morbidity is seen in 10% of patients.