Neuropsychiatric involvement in juvenile-onset systemic lupus erythematosus: Data from the UK Juvenile-onset systemic lupus erythematosus cohort study
Teresa Giani, Eve Smith, Eslam Al‐Abadi, Kate Armon, Kathryn Bailey, Coziana Ciurtin, Joyce Davidson, Janet Gardner‐Medwin, Kirsty Haslam, Dan Hawley, Alice Leahy, Valentina Leone, Flora McErlane, Devesh Mewar, Gita Modgil, Robert J. Moots, Clarissa Pilkington, Francesca Pregnolato, Athimalaipet V Ramanan, Satyapal Rangaraj, Phil Riley, Arani Sridhar, Nick Wilkinson, Rolando Cimaz, Michael W. Beresford, Christian M. Hedrich, on behalf of the UK JSLE 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 × 10 9 /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.