Neuropsychiatric involvement in juvenile-onset systemic lupus erythematosus (jSLE)

Natoli, Valentina, Charras, Amandine, Hahn, Gabriele and Hedrich, Christian M ORCID: 0000-0002-1295-6179
(2023) Neuropsychiatric involvement in juvenile-onset systemic lupus erythematosus (jSLE). MOLECULAR AND CELLULAR PEDIATRICS, 10 (1). 5-.

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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.

Item Type: Article
Uncontrolled Keywords: CNS, Inflammation, Interferon, jSLE, Juvenile, Lupus, Neurologic, Neuropsychiatric, NP-jSLE, SLE, Treatment
Divisions: Faculty of Health and Life Sciences
Faculty of Health and Life Sciences > Institute of Life Courses and Medical Sciences
Depositing User: Symplectic Admin
Date Deposited: 29 Aug 2023 08:21
Last Modified: 29 Aug 2023 08:23
DOI: 10.1186/s40348-023-00161-7
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