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一名系统性红斑狼疮患者中表现类似神经精神性狼疮的西尼罗河脑炎

West Nile encephalitis mimicking neuropsychiatric lupus in a patient with systemic lupus erythematosus.

作者信息

Iyer Neena R, McCune W Joseph, Wallace Beth I

机构信息

Department of Internal Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA.

Department of Internal Medicine, Division of Rheumatology, University of Michigan Medical School, Ann Arbor, Michigan, USA.

出版信息

BMJ Case Rep. 2019 Jul 24;12(7):e229537. doi: 10.1136/bcr-2019-229537.

Abstract

A man in his 70s with known systemic lupus erythematosus (SLE) was admitted with confusion, worsening proteinuria and cutaneous vasculitis despite adherence to his home immunosuppressive regimen. Admission laboratories were consistent with active lupus. Despite treatment with pulse-dose glucocorticoids and intravenous immunoglobulin, he developed worsening mental status and meningeal signs. Investigations revealed cerebrospinal fluid (CSF) neutrophilic and plasmacytic pleocytosis and negative cultures. Empiric treatment for SLE flare with potential neuropsychiatric involvement was continued while workup for altered mental status was ongoing. Ultimately, West Nile encephalitis was diagnosed by CSF serologies, and steroids were tapered. Altered mental status in a patient with SLE has a broad differential, and primary neuropsychiatric SLE should be considered only after exclusion of secondary causes. Although evidence of end-organ SLE activity usually lends support to a neuropsychiatric SLE diagnosis, in this case, serological and clinical evidence of SLE activity may have been triggered by acute viral infection.

摘要

一名70多岁的男性,已知患有系统性红斑狼疮(SLE),尽管坚持在家进行免疫抑制治疗,但仍因意识模糊、蛋白尿加重和皮肤血管炎入院。入院实验室检查结果与活动性狼疮相符。尽管接受了大剂量脉冲糖皮质激素和静脉注射免疫球蛋白治疗,但他的精神状态仍恶化,并出现脑膜刺激征。检查发现脑脊液(CSF)中性粒细胞和浆细胞增多,培养结果为阴性。在对精神状态改变进行检查的同时,继续对可能伴有神经精神受累的SLE发作进行经验性治疗。最终,通过脑脊液血清学检查诊断为西尼罗河脑炎,随后逐渐减少类固醇用量。SLE患者出现精神状态改变的鉴别诊断范围很广,只有在排除继发原因后才应考虑原发性神经精神性SLE。虽然终末器官SLE活动的证据通常支持神经精神性SLE的诊断,但在本例中,SLE活动的血清学和临床证据可能是由急性病毒感染引发的。

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The genetics of type I interferon in systemic lupus erythematosus.系统性红斑狼疮中 I 型干扰素的遗传学。
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