Abstract：
BACKGROUND: A symmetric leukoencephalopathy can occur in the context of systemic lupus erythematosus (SLE), often as a first manifestation of underlying rheumatologic disease. Recognition of this distinctive syndrome can prompt investigation for SLE when undiagnosed, or prompt treatment initiation when the diagnosis is already known. Earlier recognition of this syndrome could lead to more effective treatment of the disease.
METHODS: Clinical, laboratory, and radiographic features of three patients were described from an academic medical center in the United States with treatment dates between 2015 and 2022. A systematic review of literature from 1991 to 2023 yielded data for an additional 23 patients.
RESULTS: Twenty-six total patients with symmetric leukoencephalopathy were included in this study. The median age of the patients was 37 years (range 10-69), 22 patients (85 %) were female, and 4 (15 %) were male. Fourteen of 26 patients (54 %) had this as the first clinical manifestation of SLE. Contrast enhancement was present on MRI brain in 3/26 (88 %) patients. Twenty patients (77 %) were treated with pulse-dose steroids, and all but one patient received some immunomodulatory therapy. Seven patients (27 %) progressed to death. No meaningful predictive differences were found between patients who survived and those who did not.
CONCLUSIONS: In this case series and literature review patients developed symmetric leukoencephalopathy in systemic lupus erythematosus most often as the first clinical manifestation of SLE. Clinicians should consider this syndrome in any patient with acute onset of symmetric leukoencephalopathy on brain magnetic resonance imaging.
METHODS: Clinical, laboratory, and radiographic features of three patients were described from an academic medical center in the United States with treatment dates between 2015 and 2022. A systematic review of literature from 1991 to 2023 yielded data for an additional 23 patients.
RESULTS: Twenty-six total patients with symmetric leukoencephalopathy were included in this study. The median age of the patients was 37 years (range 10-69), 22 patients (85 %) were female, and 4 (15 %) were male. Fourteen of 26 patients (54 %) had this as the first clinical manifestation of SLE. Contrast enhancement was present on MRI brain in 3/26 (88 %) patients. Twenty patients (77 %) were treated with pulse-dose steroids, and all but one patient received some immunomodulatory therapy. Seven patients (27 %) progressed to death. No meaningful predictive differences were found between patients who survived and those who did not.
CONCLUSIONS: In this case series and literature review patients developed symmetric leukoencephalopathy in systemic lupus erythematosus most often as the first clinical manifestation of SLE. Clinicians should consider this syndrome in any patient with acute onset of symmetric leukoencephalopathy on brain magnetic resonance imaging.
摘要：
背景：在系统性红斑狼疮（SLE）的背景下可发生对称性白质脑病，通常作为潜在风湿病的第一表现。对这种独特的综合征的认识可以促使SLE在未诊断时进行调查，或在诊断已知时迅速开始治疗。对该综合征的早期认识可以导致对该疾病的更有效治疗。
方法：临床，实验室,3例患者的影像学特征来自美国一家学术医学中心,治疗日期为2015年至2022年.对1991年至2023年的文献进行了系统回顾，得出了另外23例患者的数据。
结果：本研究共纳入了26例对称性脑白质病患者。患者的中位年龄为37岁（范围10-69），22例（85%）为女性，4（15%）为男性。26例患者中有14例（54％）将其作为SLE的第一临床表现。3/26（88％）患者的MRI脑部存在对比增强。20例患者（77%）接受脉冲剂量类固醇治疗，除一名患者外，所有患者都接受了免疫调节治疗。7名患者（27%）进展至死亡。在存活的患者和未存活的患者之间没有发现有意义的预测差异。
结论：在本病例系列和文献综述中，系统性红斑狼疮患者最常出现对称性白质脑病，作为SLE的首发临床表现。临床医生应在脑部磁共振成像上考虑任何急性发作的对称性白质脑病患者的这种综合征。
方法：临床，实验室,3例患者的影像学特征来自美国一家学术医学中心,治疗日期为2015年至2022年.对1991年至2023年的文献进行了系统回顾，得出了另外23例患者的数据。
结果：本研究共纳入了26例对称性脑白质病患者。患者的中位年龄为37岁（范围10-69），22例（85%）为女性，4（15%）为男性。26例患者中有14例（54％）将其作为SLE的第一临床表现。3/26（88％）患者的MRI脑部存在对比增强。20例患者（77%）接受脉冲剂量类固醇治疗，除一名患者外，所有患者都接受了免疫调节治疗。7名患者（27%）进展至死亡。在存活的患者和未存活的患者之间没有发现有意义的预测差异。
结论：在本病例系列和文献综述中，系统性红斑狼疮患者最常出现对称性白质脑病，作为SLE的首发临床表现。临床医生应在脑部磁共振成像上考虑任何急性发作的对称性白质脑病患者的这种综合征。
