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.

Case Report: Rapid Progression of Cognitive Dysfunction as an Initial Feature of Systemic Lupus Erythematosus With Leukoencephalopathy: A Case Report and Literature Review

“…Similarly, 12 patients with leukoencephalopathy and NPSLE have been reported, largely involving women around a mean age of 34 years ( Table 2 ). NPSLE is more frequent in the juvenile-onset SLE than in adults with SLE ( 20 , 21 ). Moreover, nearly half of children with SLE will exhibit CNS involvement in the first year after initial diagnosis ( 20 , 21 ).…”

“…NPSLE is more frequent in the juvenile-onset SLE than in adults with SLE ( 20 , 21 ). Moreover, nearly half of children with SLE will exhibit CNS involvement in the first year after initial diagnosis ( 20 , 21 ). As shown in Table 2 , three cases that were children has been reported with leukoencephalopathy as the first manifestation of juvenile-onset NPSLE.…”

Anorexia Nervosa in Juvenile Systemic Lupus Erythematosus (SLE): A Causality Dilemma

“…Organ-system involvement in SLE can include the musculoskeletal system and the skin, the kidneys, lungs, heart, and many more [ 9 , 14 ]. Among the distinct onset subtypes, jSLE is associated with a more severe phenotype, a more diverse clinical and serological profile, and extensive organ damage [ 15 , 16 ].…”

“…NP involvement is not limited to adult-onset SLE, with 22–95% of patients having a jSLE diagnosis exhibiting NP symptoms, including hallucinations, headaches, mood disorders, stroke and weakness, cognitive impairment, anxiety, seizures, movement disorders, peripheral neuritis, transverse myelitis, or cerebrovascular disease [ 15 , 28 , 29 ]. In fact, NP is more aggressive and severe in jSLE compared to in patients with adult-onset lupus, further multiplying the morbidity for young patients [ 8 , 30 , 31 , 32 ].…”

Juvenile Neuropsychiatric Systemic Lupus Erythematosus: Identification of Novel Central Neuroinflammation Biomarkers

“…onset before age 16 years) SLE (j-SLE) is even rarer, with an estimated prevalence of 3.76/100,000 [1]. Neuropsychiatric SLE (NPSLE), defined by the presence of SLE-related neuropsychiatric (NP) involvement, has a poorly known estimated prevalence of 22 to 95% in j-SLE, largely dependent on the level of stringency in definitions [2][3][4]. NP involvement can be difficult to diagnose and raises the question as to causality i.e.…”