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

IF 2.4 Q1 PEDIATRICS Molecular and cellular pediatrics Pub Date : 2023-08-09 DOI:10.1186/s40348-023-00161-7
Valentina Natoli, Amandine Charras, Gabriele Hahn, Christian M Hedrich
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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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青少年发病的系统性红斑狼疮(jSLE)的神经精神参与。
系统性红斑狼疮(SLE)是一种罕见的自身免疫性/炎症性疾病,发病率和死亡率很高。大约15-20%的SLE患者在儿童期或青春期发病(少年型SLE/jSLE)。与成年期发病的患者相比,jSLE患者表现出更多的变异性和严重的疾病。神经精神(NP)受累是一种临床异质性和潜在的严重并发症。关于NP-jSLE发病率和患病率的报道很少,确切的病理生理机制也知之甚少。本文对现有文献进行了回顾,表明13.5-51%的jSLE患者有NP参与。在影响中枢神经系统的NP-jSLE患者中,我们提出了两个主要的亚组:(i)慢性进行性,主要是1型干扰素驱动型,对目前使用的治疗反应较差;(ii)急性侵袭型,通常在疾病早期出现,可能主要由自身反应性效应淋巴细胞介导。虽然这一假设需要在大型国际合作队列研究中进行验证,但它可能为未来的患者分层和个性化护理提供帮助。
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