Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición "Salvador Zubirán", Mexico City, Mexico.
Departmento of Neurology and Psychiatry, Instituto Nacional de Ciencias Médicas y Nutrición "Salvador Zubirán", Mexico City, Mexico.
Autoimmun Rev. 2021 Feb;20(2):102739. doi: 10.1016/j.autrev.2020.102739. Epub 2020 Dec 14.
Posterior Reversible Encephalopathy Syndrome (PRES) is an acute neurological syndrome clinically characterized by seizures, altered mental status, headache, and visual disturbances. It is caused by a variety of abnormalities in the endothelial function that ultimately result in vasogenic edema in the circulation of the central nervous system. This is reflected by the neuroimaging findings, that most often show reversible parieto-occipital edema. An important proportion of patients with PRES present with Systemic Lupus Erythematosus (SLE), and its complications, as their sole risk factors. This review describes the relationship between these two clinical entities and explains the pathophysiological models that have been proposed to describe the development of PRES. We explain how SLE can cause alterations in every pathway implicated in the development of PRES. Given the relatively high frequency and the distinct clinical course, PRES in the setting of SLE might be best described as a distinct neuropsychiatric syndrome associated with SLE.
后部可逆性脑病综合征(PRES)是一种急性神经系统综合征,临床上以癫痫发作、精神状态改变、头痛和视觉障碍为特征。它是由内皮功能的各种异常引起的,最终导致中枢神经系统循环中的血管源性水肿。神经影像学检查结果反映了这一点,最常见的是可逆性顶枕叶水肿。相当一部分 PRES 患者以系统性红斑狼疮(SLE)及其并发症为唯一危险因素。本综述描述了这两种临床实体之间的关系,并解释了已提出的描述 PRES 发展的病理生理模型。我们解释了 SLE 如何导致 PRES 发展过程中涉及的每条途径的改变。鉴于其相对较高的频率和独特的临床病程,SLE 中的 PRES 最好被描述为一种与 SLE 相关的独特神经精神综合征。