医学
发病机制
系统性红斑狼疮
免疫学
自身抗体
神经免疫学
认知
免疫系统
神经科学
重症监护医学
生物信息学
精神科
心理学
病理
疾病
抗体
生物
作者
Noa Schwartz,Ariel Stock,Chaim Putterman
标识
DOI:10.1038/s41584-018-0156-8
摘要
Patients with systemic lupus erythematosus (SLE) frequently show symptoms of central nervous system (CNS) involvement, termed neuropsychiatric SLE (NPSLE). The CNS manifestations of SLE are diverse and have a broad spectrum of severity and prognostic implications. Patients with NPSLE typically present with nonspecific symptoms, such as headache and cognitive impairment, but might also experience devastating features, such as memory loss, seizures and stroke. Some features of NPSLE, in particular those related to coagulopathy, have been characterized and an evidence-based treatment algorithm is available. The cognitive and affective manifestations of NPSLE, however, remain poorly understood. Various immune effectors have been evaluated as contributors to its pathogenesis, including brain-reactive autoantibodies, cytokines and cell-mediated inflammation. Additional brain-intrinsic elements (such as resident microglia, the blood–brain barrier and other neurovascular interfaces) are important facilitators of NPSLE. As yet, however, no unifying model has been found to underlie the pathogenesis of NPSLE, suggesting that this disease has multiple contributors and perhaps several distinct aetiologies. This heterogeneity presents a challenge for clinicians who have traditionally relied on empirical judgement in choosing treatment modalities for patients with NPSLE. Improved understanding of this manifestation of SLE might yield further options for managing this disease. Neuropsychiatric systemic lupus erythematosus (NPSLE) remains highly challenging to diagnose and treat. This Review describes current understanding of its pathogenesis, along with novel therapies and diagnostic tools that could eventually improve the management of NPSLE.
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