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Open Access 01-12-2023 | Systemic Lupus Erythematosus | Review

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

Authors: Valentina Natoli, Amandine Charras, Gabriele Hahn, Christian M. Hedrich

Published in: Molecular and Cellular Pediatrics | Issue 1/2023

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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Title: Neuropsychiatric involvement in juvenile-onset systemic lupus erythematosus (jSLE)
Authors: Valentina Natoli
Amandine Charras
Gabriele Hahn
Christian M. Hedrich
Publication date: 01-12-2023
Publisher: Springer International Publishing
Keywords: Systemic Lupus Erythematosus
Interferon
Vasculitis
Vasculitis
Published in: Molecular and Cellular Pediatrics / Issue 1/2023
Electronic ISSN: 2194-7791
DOI: https://doi.org/10.1186/s40348-023-00161-7

Keynote webinar | Spotlight on medication adherence

Springer Medicine

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

Abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

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