Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, South Korea.
Lupus. 2013 Aug;22(9):885-91. doi: 10.1177/0961203313496341. Epub 2013 Jul 11.
Posterior reversible encephalopathy syndrome (PRES) is an uncommon neurologic condition associated with systemic lupus erythematosus (SLE). This study aimed to demonstrate the risk factors and clinical outcome of PRES in patients with SLE. Fifteen patients with SLE were diagnosed with PRES by characteristic clinical manifestations and magnetic resonance imaging (MRI) features from 2000 to 2012. Clinical profiles and outcomes were assessed for this study population. Additionally, 48 SLE patients with neurologic symptoms who underwent brain MRI were included for comparative analyses. The median age and duration of SLE in patients with PRES was 27 and 6.1 years, respectively. Comparison between patients with and without PRES revealed significant differences in the presentation of hypertension and seizure, lupus nephritis with renal insufficiency, treatment with high-dose steroid and cyclophosphamide, recent transfusion, and lupus activity measured by SLE disease activity index. Renal failure was the single independent factor with a high odds ratio of 129.250 by multivariate analysis. Of 15 patients, four experienced relapse and two died of sepsis during hospitalization. Our results suggest that lupus nephritis with renal dysfunction and other related clinical conditions can precede the occurrence of PRES in patients with SLE. It is important to perform early brain imaging for a timely diagnosis of PRES when clinically suspected.
后部可逆性脑病综合征（PRES）是一种与系统性红斑狼疮（SLE）相关的不常见神经系统疾病。本研究旨在探讨 SLE 患者 PRES 的危险因素和临床转归。2000 年至 2012 年间，通过特征性临床表现和磁共振成像（MRI）特征，对 15 例 SLE 患者诊断为 PRES。对该研究人群进行了临床特征和转归评估。此外，还纳入了 48 例有神经系统症状且接受脑 MRI 检查的 SLE 患者进行对比分析。PRES 患者的中位年龄和 SLE 病程分别为 27 岁和 6.1 年。患有 PRES 和不患有 PRES 的患者之间的比较显示，高血压和癫痫、狼疮肾炎合并肾功能不全、大剂量类固醇和环磷酰胺治疗、近期输血和 SLE 疾病活动度（通过 SLE 疾病活动指数测量）的出现存在显著差异。多变量分析显示，肾功能衰竭是唯一具有高比值比（OR）的独立因素（OR=129.250）。在 15 例患者中，4 例复发，2 例因脓毒症在住院期间死亡。我们的研究结果表明，狼疮肾炎合并肾功能不全和其他相关临床情况可能先于 SLE 患者 PRES 的发生。当临床上怀疑 PRES 时，及时进行脑影像学检查以做出早期诊断非常重要。
