Hershko Alon Y, Berkun Yackov, Mevorach Dror, Rubinow Alan, Naparstek Yaakov
Clinical Immunology and Rheumatology Units, Department of Medicine Hadassah University Hospital, Jerusalem, Israel.
Semin Arthritis Rheum. 2008 Oct;38(2):110-5. doi: 10.1016/j.semarthrit.2007.09.007. Epub 2008 Jan 25.
To assess whether increased intracranial pressure (IIP) is associated or coincidental with systemic lupus erythematosus (SLE) and to characterize the patients with this condition, its commonly used treatment, and outcome.
A retrospective cohort study was conducted at the Hadassah Medical Center, Jerusalem, Israel. Files were retrieved from the hospital archives by screening records from 1980 to 2006 using the terms "SLE" and "pseudotumor cerebri" or "benign intracranial pressure." Medical records were screened for data regarding: (1) The prevalence of IIP among hospitalized patients; (2) the demographic, clinical, and laboratory characteristics of SLE patients reported to have at least 1 episode of IIP.
Ten of 651 hospitalized SLE patients (prevalence, 1.5%) were found to have IIP, exceeding the prevalence of 1 to 19 per 100,000 reported in the general population. In 3 patients this was a presenting manifestation of SLE and the average time elapsed until IIP diagnosis was 10.7 months. Arabs were strikingly overrepresented among the 10 individuals, comprising 6 patients (versus 19% Arabs of all SLE patients). In 8 patients, other central nervous system (CNS) abnormalities were found either clinically or in laboratory tests. Two individuals had elevated antiphospholipid antibodies (aPL) and a history of thromboembolic events, compatible with antiphopholipid syndrome. The most commonly employed treatments were corticosteroids and acetazolamide, each in 9 patients with generally good clinical response.
We propose that IIP is a manifestation of SLE and that corticosteroids should be considered as first-line treatment.
评估颅内压升高（IIP）与系统性红斑狼疮（SLE）是相关还是并存，并描述患有这种情况的患者、其常用治疗方法及预后。
在以色列耶路撒冷的哈达萨医疗中心进行了一项回顾性队列研究。通过筛查1980年至2006年的医院档案记录，使用“SLE”和“假性脑瘤”或“良性颅内压”等术语检索文件。对病历进行筛查以获取以下数据：（1）住院患者中IIP的患病率；（2）报告至少有1次IIP发作的SLE患者的人口统计学、临床和实验室特征。
651例住院SLE患者中有10例（患病率为1.5%）被发现患有IIP，超过了普通人群中每10万人1至19例的患病率。在3例患者中，这是SLE的首发表现，直到IIP诊断的平均时间为10.7个月。在这10例患者中，阿拉伯人明显占比过高，有6例患者（而所有SLE患者中阿拉伯人占19%）。在8例患者中，临床或实验室检查发现有其他中枢神经系统（CNS）异常。2例患者抗磷脂抗体（aPL）升高且有血栓栓塞事件史，符合抗磷脂综合征。最常用的治疗方法是皮质类固醇和乙酰唑胺，各有9例患者使用，总体临床反应良好。
我们认为IIP是SLE的一种表现，皮质类固醇应被视为一线治疗药物。
