Background Neuropsychiatric involvement in systemic lupus erythematosus (NPSLE) includes the neurological syndromes of the central, peripheral or autonomic nervous systems and psychiatric disorders. Some studies suggests that juvenile systemic lupus erythematosus (jSLE) has a more serious course and a more frequent neuropsychiatric involvement than adult onset, coursing with significant morbidity and mortality. Its prevalence ranges from 22 to 50.9%.
Objectives To assess neuropsychiatric manifestations in juvenile-onset SLE patients and study the predictors and association to disease characteristics.
Methods Retrospective observational study was performed including consecutive patients with jSLE (disease onset before 16 years of age,) that fulfilled American College of Rheumatology (ACR) criteria followed in our Paediatric Rheumatology Unit. Clinical, demographic and laboratory characteristics were retrospectively collected by consulting the medical records. The neuropsychiatric manifestations were defined according to the nomenclature and classification of ACR. Patients with NPSLE were compared with others using Student t-test, Mann-Whitney test, Chi-square or Fisher test (SPSS 23.0). Significance level was set as <0.05.
Results 38 patients were included, 92.1% (35) were female, with a mean age at diagnosis of 12.9 ± 3 years. Median period between onset of symptoms and diagnosis of SLE was 0.3 [0–1.2] years and median duration of follow-up was 14 [0.75–26].
Neuropsychiatric manifestations of the SLE were observed in 8 (21%) patients: chorea (n=1), psychosis (n=2), seizures (n=1), transverse myelitis (1), stroke and weakness (n=1), headache (n=2).
Among the 8 patients with NPSLE, 7 were female and 1 was male. The median time between diagnosis and NPSLE manifestation was 1.75 [0–3] years. The two patients with headaches were treated with oral glucocorticoid. The remaining patients were treated methylprednisole pulses, 3 were treated with cyclophosphamide and 1 with rituximab.
Full recovery was observed in almost patients, except for those with headaches that had many recurrences of symptoms during follow up.
Comparing patients that had NPSLE with the remaining, we found a statistically significant higher SLEDAI and SLICC scores in those with NPSLE (4 vs 2, p=00.04 and 1.5 vs 0, p=0.003, respectively).The age, sex and disease duration were comparable between the groups.
NPSLE was associated with more frequent lupus nephritis (75% vs 43.3%, p=0.03) and presence of antiphospholipide syndrome (APS) (20% vs 11.2%, p=0.04).
NPSLE patients also had higher lupus anti-coagulant prevalence (25% vs 10.7%) and anticardiolipine antibodies (23.3% vs 12.5%) %), yet none of the above reached statistical significance.
Conclusions The neuropsychiatric manifestations are frequently observed in patients with jSLE and central nervous system manifestations were more frequently observed than manifestations affecting the peripheral nervous system. Our study suggests an association between NPSLE and lupus nephritis, higher disease activity and the presence of APS. We observed a good outcome with complete resolution in the majority of the cases.
Disclosure of Interest None declared