Department of Medical Sciences, Rheumatology Unit, University of Ferrara and Azienda Ospedaliero-Universitaria Sant'Anna, Cona, Ferrara, Italy.
Department of Medical Sciences, Rheumatology Unit, University of Ferrara and Azienda Ospedaliero-Universitaria Sant'Anna, Cona, Ferrara, Italy.
J Autoimmun. 2016 Nov;74:41-72. doi: 10.1016/j.jaut.2016.06.013. Epub 2016 Jul 12.
Neuropsychiatric (NP) involvement in Systemic Lupus Erythematosus (SLE), can be a severe and troubling manifestation of the disease that heavily impacts patient's health, quality of life and disease outcome. It is one of the most complex expressions of SLE which can affect central, peripheral and autonomous nervous system. Complex interrelated pathogenetic mechanisms, including genetic factors, vasculopathy, vascular occlusion, neuroendocrine-immune imbalance, tissue and neuronal damage mediated by autoantibodies, inflammatory mediators, blood brain barrier dysfunction and direct neuronal cell death can be all involved. About NPSLE a number of issues are still matter of debate: from classification and burden of NPSLE to attribution and diagnosis. The role of neuroimaging and new methods of investigation still remain pivotal and rapidly evolving as well as is the increasing knowledge in the pathogenesis. Overall, two main pathogenetic pathways have been recognized yielding different clinical phenotypes: a predominant ischemic-vascular one involving large and small blood vessels, mediated by aPL, immune complexes and leuko-agglutination which it is manifested with more frequent focal NP clinical pictures and a predominantly inflammatory-neurotoxic one mediated by complement activation, increased permeability of the BBB, intrathecal migration of autoantibodies, local production of immune complexes and pro-inflammatory cytokines and other inflammatory mediators usually appearing as diffuse NP manifestations. In the attempt to depict a journey throughout NPSLE from diagnosis to a reasoned therapeutic approach, classification, epidemiology, attribution, risk factors, diagnostic challenges, neuroimaging techniques and pathogenesis will be considered in this narrative review based on the most relevant and recent published data.
神经精神性（NP）狼疮累及系统性红斑狼疮（SLE）是一种严重且令人困扰的疾病表现，严重影响患者的健康、生活质量和疾病结局。它是 SLE 最复杂的表现之一，可以影响中枢、周围和自主神经系统。复杂的相互关联的发病机制，包括遗传因素、血管病变、血管闭塞、神经内分泌-免疫失衡、自身抗体、炎症介质、血脑屏障功能障碍和直接神经元细胞死亡介导的组织和神经元损伤，都可能参与其中。关于 NPSLE，仍有许多问题存在争议：从 NPSLE 的分类和负担到归因和诊断。神经影像学和新的研究方法的作用仍然至关重要且快速发展，同时发病机制的知识也在不断增加。总的来说，已经认识到两种主要的发病途径，产生不同的临床表型：一种主要的缺血性血管途径，涉及大血管和小血管，由 aPL、免疫复合物和白细胞凝集介导，表现为更频繁的局灶性 NP 临床症状；另一种主要的炎症性神经毒性途径，由补体激活、BBB 通透性增加、自身抗体鞘内迁移、局部产生免疫复合物和促炎细胞因子和其他炎症介质介导，通常表现为弥漫性 NP 表现。在尝试描绘从诊断到合理治疗方法的 NPSLE 之旅中，我们将根据最新的相关数据，在这篇叙述性综述中考虑分类、流行病学、归因、危险因素、诊断挑战、神经影像学技术和发病机制。
