Centre National de la Recherche Scientifique (CNRS), Immunopathologie et Chimie Thérapeutique/Laboratory of Excellence Medalis, Institut de Biologie Moléculaire et Cellulaire, 15 rue René Descartes, 67000 Strasbourg, France.
Nat Rev Neurol. 2014 Oct;10(10):579-96. doi: 10.1038/nrneurol.2014.148. Epub 2014 Sep 9.
Systemic lupus erythematosus (SLE) is a complex clinical syndrome, elements of which remain poorly understood. Although recognized over 140 years ago when Kaposi recorded the systemic nature and manifestations of the disease, CNS involvement represents one of the least understood aspects of SLE. This knowledge gap remains despite the fact that up to 75% of adults and children with SLE will, at some point over the course of the disease and to different extents, experience the various disabling effects of neuropsychiatric SLE (NPSLE). Indeed, after decades of research, our understanding of the underlying pathophysiology of NPSLE, in particular, remains limited. Numerous factors contribute to the immune dysfunction that occurs in SLE, including genetic, environmental and hormonal influences, and the contributory or predisposing components that lead to neurological tropism of disease in some patients have not been clearly demonstrated. Features of NPSLE pathogenesis that might be directly linked to clinical manifestations have been identified; however, the complexity and variety of NPSLE symptoms and the clinical overlap with other psychiatric disorders continue to make accurate diagnosis difficult and time-consuming. Thus, efforts to define biomarkers of NPSLE are needed to improve prediction of disease outcomes and guide treatment. In this article, we review the manifestation and pathogenesis of NPSLE, focusing on the features that might aid identification of potential biomarkers.
系统性红斑狼疮（SLE）是一种复杂的临床综合征，其许多方面仍未被充分理解。尽管早在 140 多年前 Kaposi 就记录了这种疾病的系统性特征和表现，但中枢神经系统（CNS）受累是 SLE 中了解最少的方面之一。尽管高达 75%的成人和儿童 SLE 患者在疾病过程中的某个时刻，且在不同程度上，会经历神经精神性 SLE（NPSLE）的各种致残影响，但这一知识差距仍然存在。事实上，经过几十年的研究，我们对 NPSLE 的潜在病理生理学的理解仍然有限。许多因素导致 SLE 中发生免疫功能障碍，包括遗传、环境和激素影响，并且尚未明确证明导致疾病在某些患者中具有神经趋向性的促成或易患成分。已经确定了可能与临床表现直接相关的 NPSLE 发病机制特征；然而，NPSLE 症状的复杂性和多样性以及与其他精神障碍的临床重叠仍然使得准确诊断变得困难且耗时。因此，需要努力定义 NPSLE 的生物标志物，以改善疾病结局的预测并指导治疗。在本文中，我们综述了 NPSLE 的表现和发病机制，重点关注可能有助于确定潜在生物标志物的特征。
