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

UK JSLE Cohort Study, Teresa Giani*, Eve MD Smith, Eslam Al-Abadi, Kate Armon, Kathryn Bailey, Coziana Ciurtin, Joyce Davidson, Janet Gardner-Medwin, Kirsty Haslam, Dan P Hawley, Alice Leahy, Valentina Leone, Flora McErlane, Devesh Mewar, Gita Modgil, Robert Moots, Clarissa Pilkington, Francesca Pregnolato, Athimalaipet V RamananSatyapal Rangaraj, Phil Riley, Arani Sridhar, Nick Wilkinson, Rolando Cimaz, Michael W Beresford, Christian M Hedrich

*Corresponding author for this work

Research output: Contribution to journalArticle (Academic Journal)peer-review

25 Citations (Scopus)

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.
Original languageEnglish
Pages (from-to)1955-1965
Number of pages11
JournalLupus
Volume30
Issue number12
Early online date2 Oct 2021
DOIs
Publication statusPublished - Oct 2021

Bibliographical note

Publisher Copyright:
© The Author(s) 2021.

Keywords

  • Adolescent
  • Child
  • Cohort Studies
  • Female
  • Humans
  • Lupus Erythematosus, Systemic/complications
  • Lupus Vasculitis, Central Nervous System/epidemiology
  • Male
  • Mental Disorders/etiology
  • United Kingdom/epidemiology

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