Date: Monday, November 9, 2020
Session Type: Poster Session D
Session Time: 9:00AM-11:00AM
Background/Purpose: Juvenile-onset systemic lupus erythematosus (JSLE) is a rare autoimmune/inflammatory disease, accounting for up to 20% of SLE cases. Though clinically similar to adult-onset disease, it frequently follows a more severe course. Neuropsychiatric (NP) involvement in JSLE can be aggressive and significantly affect patients’ quality of life as well as disease outcomes.
The aim of this study was to describe the demographic characteristics, clinical and laboratory features of NP involvement in JSLE.
Methods: We analyzed data from JSLE patients enrolled in the UK JSLE Cohort Study between August 2006 and June 2019. Demographic (age, gender, ethnicity, family history), clinical (1997 ACR classification criteria, disease activity BILAG, SLICC, and damage index SLICC-SDI) and laboratory (ESR, CRP, CBC with diff, ANA, anti-ENA, anti-dsDNA, aCL, lipid profile, renal function, C3, C4, Ig levels, thyroid function, UA) data collected at disease onset and at last visit were analyzed.
Results: A total of 428 JSLE patients were included, with a female:male ratio of 5.4:1. The median age at diagnosis was 12.2 years (range: 0-17). A majority of JSLE patients were Caucasian (51.4%), followed by patients of South Asian (23.3%), Black African/Caribbean (16.7%), and East Asian (6.5%) descent. Patients with headaches as the only NP symptom were excluded here, because of the low specificity of this feature.
Overall, one quarter of JSLE patients (107/428, 25%) showed NP features; in 48.5% of these cases, NP symptoms were the presenting manifestation. The median age at disease onset and ethnic composition did not differ between sub-cohorts with vs without NP involvement. Most frequently recorded NP manifestations included cognitive impairment (n=45, 42%), seizures (n=21, 20%), psychotic features (n=11, 10%), peripheral nerve involvement (n=9, 8%), cerebral vasculitis (n=10, 9%), and ischaemic stroke (n=7, 6%). Headache was an accompanying manifestation in 74% of all NP-JSLE patients.
While no differences were recorded in autoantibody patterns and immune cell counts, lower platelet counts (< 100.000/mmc) were found in patients with NP involvement (p=0.02). Children with NP involvement showed both a higher number of ACR criteria (mean 4.9 vs 4.6, p=0.07) and higher SLICC scores (0.3 vs 0.2, p=0.029) at disease onset.
As compared to JSLE patients without neurological involvement, at diagnosis, patients with NP-JSLE exhibited higher disease activity (pBILAG) in the constitutional (p< 0.01) and ophthalmologic (p< 0.05) domains. At last visit, NP-JSLE patients had an increased number of ACR criteria (mean 6 vs 5.5; p< 0.001) and more damage (SLICC) (mean 1.2 vs 0.4; p< 0.001). Comparing JSLE patients with primary NP involvement to individuals who developed NP symptoms later, patients with early NP involvement exhibited more damage (SLICC) at diagnosis (mean 0.63 vs 0.05; p< 0.01) and last visit (mean 1.7 vs 0.7; p< 0.01).
Conclusion: Approximately 25% of JSLE patients enrolled in the UK JSLE Cohort Study have NP involvement. Patients with NP involvement exhibit higher disease activity and more disease-associated damage when compared to patients without NP involvement.
To cite this abstract in AMA style:Giani T, Smith E, Cimaz R, Beresford M, Hedrich C. Neuropsychiatric Involvement in Juvenile-onset Systemic Lupus Erythematosus (JSLE): Data from the UK JSLE Cohort Study [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 10). https://acrabstracts.org/abstract/neuropsychiatric-involvement-in-juvenile-onset-systemic-lupus-erythematosus-jsle-data-from-the-uk-jsle-cohort-study/. Accessed November 27, 2021.
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