Section of Rheumatology, University of Chicago Medical Center, IL, USA.
J Clin Rheumatol. 2013 Sep;19(6):334-40. doi: 10.1097/RHU.0b013e3182a21ffd.
BACKGROUND/OBJECTIVE: Posterior reversible encephalopathy syndrome (PRES) is an underrecognized and reversible condition in systemic lupus erythematosus (SLE) that could mimic neuropsychiatric lupus. Identification of any distinct clinical patterns is important as one would need to escalate rather than decrease or discontinue immune suppression in neuropsychiatric lupus.
We retrospectively identified and described 5 patients with SLE who were hospitalized and diagnosed with PRES from 2008 to 2013 in a tertiary medical center and reviewed relevant literature.
Posterior reversible encephalopathy syndrome in SLE occurred in young women with age distribution from 19 to 37 years. At the time of presentation, all had hypertension (systolic blood pressures ranging from 150 to 220), moderate to severe disease activity (Systemic Lupus Erythematosus Disease Activity Index scores ranging from 11 to 41), and prototypical magnetic resonance imaging findings of PRES and nephritis (4 of 5 patients had biopsy-proven lupus nephritis). Seizures, headache, and confusion were the most common clinical symptoms. One patient had intracerebral hematoma, and 2 patients had cerebral petechial hemorrhages. All patients improved without any neurological deficits, with a mean hospital stay of 11.2 days.
Systemic lupus erythematosus should be considered in the differential diagnosis of patients who present with PRES. One should have a low threshold for magnetic resonance imaging especially when neurological symptoms occur in young women with or without an established diagnosis of SLE and especially among those with active SLE, lupus nephritis, renal failure, and/or poorly controlled hypertension. Given the good prognosis of PRES in SLE patients with early supportive treatment, prompt recognition is crucial to institute appropriate management.
背景/目的：后部可逆性脑病综合征（PRES）是系统性红斑狼疮（SLE）中一种认识不足且可逆转的病症，可能类似于神经精神狼疮。识别任何独特的临床模式都很重要，因为在神经精神狼疮中，人们需要增强而不是减少或停止免疫抑制。
我们回顾性地确定并描述了 5 名于 2008 年至 2013 年在一家三级医疗中心住院并被诊断为 PRES 的 SLE 患者，并回顾了相关文献。
SLE 中的 PRES 发生在年龄在 19 至 37 岁之间的年轻女性中。在出现时，所有患者均有高血压（收缩压范围为 150 至 220mmHg）、中重度疾病活动（系统性红斑狼疮疾病活动指数评分范围为 11 至 41），以及 PRES 和肾炎的典型磁共振成像表现（4/5 名患者经活检证实有狼疮肾炎）。最常见的临床症状是癫痫发作、头痛和意识混乱。1 名患者有颅内血肿，2 名患者有脑点状出血。所有患者均无神经功能缺损而改善，平均住院时间为 11.2 天。
在出现 PRES 的患者中，应考虑系统性红斑狼疮作为鉴别诊断。当出现神经系统症状的年轻女性（无论是否有明确的 SLE 诊断），尤其是在那些有活动性 SLE、狼疮肾炎、肾衰竭和/或未得到良好控制的高血压的患者中，应降低对磁共振成像的门槛。鉴于 PRES 在 SLE 患者中早期支持治疗的预后良好，及时识别对于实施适当的治疗至关重要。
