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-.
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.
Item Type: | Article |
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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 |
Open Access URL: | https://molcellped.springeropen.com/articles/10.11... |
Related URLs: | |
URI: | https://livrepository.liverpool.ac.uk/id/eprint/3172354 |