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Abstract
Systemic lupus erythematosus (SLE) is a rare autoimmune/inflammatory disease with significant morbidity and mortality. Approximately 15–20% of SLE patients develop the disease during childhood or adolescence (juvenile-onset SLE/jSLE). Patients with jSLE exhibit more variable and severe disease when compared to patients with disease-onset during adulthood. Neuropsychiatric (NP) involvement is a clinically heterogenous and potentially severe complication. Published reports on the incidence and prevalence of NP-jSLE are scarce, and the exact pathophysiology is poorly understood.
This manuscript provides a review of the existing literature, suggesting NP involvement in 13.5–51% of jSLE patients. Among patients with NP-jSLE affecting the CNS, we propose two main subgroups: (i) a chronic progressive, predominantly type 1 interferon-driven form that poorly responds to currently used treatments, and (ii) an acutely aggressive form that usually presents early during the disease that may be primarily mediated by auto-reactive effector lymphocytes. While this hypothesis requires to be tested in large collaborative international cohort studies, it may offer future patient stratification and individualised care.
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1 Institute of Life Course and Medical Sciences, University of Liverpool, Department of Women’s and Children’s Health, Liverpool, UK (GRID:grid.10025.36) (ISNI:0000 0004 1936 8470); Institute in the Park, Alder Hey Children’s NHS Foundation Trust, Department of Rheumatology, Liverpool, UK (GRID:grid.417858.7) (ISNI:0000 0004 0421 1374); Università degli Studi di Genova, Dipartimento di Neuroscienze, Riabilitazione, Oftalmologia, Genetica e Scienze Materno-Infantili, Genoa, Italy (GRID:grid.5606.5) (ISNI:0000 0001 2151 3065)
2 Institute of Life Course and Medical Sciences, University of Liverpool, Department of Women’s and Children’s Health, Liverpool, UK (GRID:grid.10025.36) (ISNI:0000 0004 1936 8470)
3 Universitätsklinikum Carl Gustav Carus, Dresden University of Technology, Department of Radiology, Dresden, Germany (GRID:grid.412282.f) (ISNI:0000 0001 1091 2917)
4 Institute of Life Course and Medical Sciences, University of Liverpool, Department of Women’s and Children’s Health, Liverpool, UK (GRID:grid.10025.36) (ISNI:0000 0004 1936 8470); Institute in the Park, Alder Hey Children’s NHS Foundation Trust, Department of Rheumatology, Liverpool, UK (GRID:grid.417858.7) (ISNI:0000 0004 0421 1374)