Neuropsychiatric involvement in juvenile-onset systemic lupus erythematosus: Data from the UK Juvenile-onset systemic lupus erythematosus cohort study

Abstract

Introduction: Juvenile-onset systemic lupus erythematosus (JSLE) is a rare autoimmune/inflammatory disease with significant morbidity and mortality. Neuropsychiatric (NP) involvement is a severe complication, encompassing a heterogeneous range of neurological and psychiatric manifestations.

Methods: Demographic, clinical, and laboratory features of NP-SLE were assessed in participants of the UK JSLE Cohort Study, and compared to patients in the same cohort without NP manifestations.

Results: A total of 428 JSLE patients were included in this study, 25% of which exhibited NP features, half of them at first visit. Most common neurological symptoms among NP-JSLE patients included headaches (78.5%), mood disorders (48.6%), cognitive impairment (42%), anxiety (23.3%), seizures (19.6%), movement disorders (17.7%), and cerebrovascular disease (14.9%). Peripheral nervous system involvement was recorded in 7% of NP-SLE patients. NP-JSLE patients more frequently exhibited thrombocytopenia (<100 × 10⁹/L) (p = 0.04), higher C-reactive protein levels (p = 0.01), higher global pBILAG score at first visit (p < 0.001), and higher SLICC damage index score at first (p = 0.02) and last (p < 0.001) visit when compared to JSLE patients without NP involvement.

Conclusions: A significant proportion of JSLE patients experience NP involvement (25%). Juvenile-onset NP-SLE most commonly affects the CNS and is associated with increased overall disease activity and damage.

Keywords: Systemic lupus erythematosus; central nervous system; juvenile; neuropsychiatric; pediatric; peripheral nervous system.

Figure 1.

Frequency of neuropsychiatric syndromes. Distribution of each neuropsychiatric syndrome in the present series among the 19 standardized neuropsychiatric syndromes linked to systemic lupus erythematosus identified by the American College of Rheumatology ad hoc Committee. Peripheral, neuropsychiatric feature involving the peripheral nervous system. “Others” include: acute confusional state, aseptic meningitis, Guillain Barrè syndrome, and myelopathy that individually represent less than 2.5% of all manifestations.

Figure 2.

Significant differences in laboratory findings in juvenile-onset systemic lupus erythematosus patients with or without neuropsychiatric involvement. Neuropsychiatric, NP; Systemic lupus erythematosus, SLE; CRP, C-reactive protein. Low platelet numbers <100 × 10⁹/L. For continuous variables, Mann–Whitney U test was used; for categorical variables, Chi-square test was used.