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Neuropsychiatric involvement in juvenile-onset systemic lupus erythematosus: Data from the UK Juvenile-onset systemic lupus erythematosus cohort study. 2021
Teresa Giani, and
Eve Md Smith, and
Eslam Al-Abadi, and
Kate Armon, and
Kathryn Bailey, and
Coziana Ciurtin, and
Joyce Davidson, and
Janet Gardner-Medwin, and
Kirsty Haslam, and
Dan P Hawley, and
Alice Leahy, and
Valentina Leone, and
Flora McErlane, and
Devesh Mewar, and
Gita Modgil, and
Robert Moots, and
Clarissa Pilkington, and
Francesca Pregnolato, and
Athimalaipet V Ramanan, and
Satyapal Rangaraj, and
Phil Riley, and
Arani Sridhar, and
Nick Wilkinson, and
Rolando Cimaz, and
Michael W Beresford, and
Christian M Hedrich, and
Rheumatology Unit, AOU Meyer, Florence, Italy.
BACKGROUND 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.
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