Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with significant morbidity and mortality. Approximately 15-20% of SLE patients develop disease before their 16th birthday and are diagnosed with juvenile-onset SLE (JSLE). Neuropsychiatric (NP) involvement is a severe complication, encompassing neurological and psychiatric manifestations.
This study aimed to investigate the prevalence, demographic characteristics, and clinical features of NP disease in JSLE patients enrolled in the UK JSLE Cohort Study. Furthermore, associations between NP involvement and other clinical and/or laboratory features, as well correlation with disease severity and outcomes were investigated.
Demographic, clinical and laboratory features of NP-SLE of a large national cohort of JSLE were collected and compared to patients in the same cohort without NP manifestations.
A total of 428 JSLE patients were included in this study, 25% of who 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 x 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 visit (p<0.001) when compared to JLSE patients without NP involvement.
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.