探讨儿童系统性红斑狼疮(cSLE)合并颅内压增高(IH)患儿的临床特征、诊疗策略及预后。

选择2015年1月至2025年9月首都医科大学附属首都儿童医学中心收治的7例cSLE合并IH患儿(患儿1~7)为研究对象。采用回顾性分析方法对其临床资料，包括一般情况、临床表现、实验室检查、影像学特征、治疗及随访结果进行统计学分析。以"系统红斑狼疮""颅内压增高""儿童"为中文关键词，以"systemic lupus erythematosus""intracranial hypertension""child"为英文关键词，在万方数据知识服务平台、中国知网数据库、维普中文期刊服务平台、PubMed及Web of Science数据库中，检索cSLE合并IH患儿的相关研究文献。本次文献检索时限设定为各数据库建库至2025年11月30日。本研究遵循的程序符合首都医科大学附属首都儿童医学中心医学伦理委员会规定，通过该伦理委员会审批(审批文号：SHERLLM2025008)。

①本研究患儿1~7均为女性，中位发病年龄为13岁(9~15岁)，均处于cSLE重度活动期[系统性红斑狼疮疾病活动指数2000(SLEDAI-2000)评分中位数为21分(17~33分)]。所有患儿均出现头痛症状，部分伴头晕、呕吐或视觉异常，肥胖或超重患儿为3例。其实验室检查结果显示，抗ds-DNA抗体呈阳性为6例，抗核糖体P蛋白抗体呈阳性为4例，抗磷脂抗体呈阳性为3例，补体水平普遍较正常值降低。多模式影像学检查结果无特异性改变。所有患儿均接受甲泼尼龙冲击治疗(MPPT)联合免疫抑制剂治疗，其中6例联合生物制剂治疗，同时予甘露醇等降颅压治疗。对患儿1~7出院后的中位随访时间为19个月(12~62个月)，均获得持续缓解，未见复发。②根据本研究设定的文献检索策略，共检索到关于cSLE合并IH患儿的研究文献为8篇，纳入cSLE合并IH患儿为15例，加上本研究的患儿1~7，共计22例cSLE合并IH患儿被纳入进行以下综合分析的结果显示，其中女性患儿为21例(95.5%)，中位发病年龄为13岁，合并超重或肥胖为10例(45.5%)；6例(27.3%)患儿的IH诊断早于cSLE确诊；11例(50.0%)患儿伴有视盘水肿；IH确诊时，15例报道SLE活动度评分者，均处于SLE重度活动期，SLEDAI-2000评分的中位数为31分(17~50分)；实验室检查结果提示，22例cSLE合并IH患儿中，抗磷脂抗体呈阳性者为9例(40.9%)，抗核糖体P蛋白抗体呈阳性者为8例(36.4%)。

cSLE合并IH好发于青春期女性，常伴肥胖或超重，多在cSLE高度活动期出现，部分患儿可伴抗核糖体P蛋白抗体和(或)抗磷脂抗体呈阳性。部分cSLE合并IH患儿的IH可先于cSLE被确诊，而且无视盘水肿表现，易被临床漏诊。若cSLE合并IH患儿出现明显视盘水肿，则需警惕脑疝风险。

To investigate the clinical characteristics, management strategies, and prognosis of intracranial hypertension (IH) in children with systemic lupus erythematosus (cSLE).

A retrospective analysis was conducted on 7 children with cSLE complicated by IH who were admitted to Capital Center for Children′s Health, Capital Medical University, from January 2015 to September 2025. Clinical data including demographic characteristics, clinical manifestations, laboratory findings, imaging features, treatment, and follow-up outcomes were collected and analyzed. In addition, a literature review was performed by searching Wanfang Data, China National Knowledge Infrastructure (CNKI), VIP database, PubMed, and Web of Science using the keywords " systemic lupus erythematosus" " intracranial hypertension" and " child". The search covered the period from database inception to November 30, 2025. This study was approved by the institutional ethics committee (Approval No. SHERLLM2025008).

① All 7 patients were female with a median age of 13 years (range: 9-15 years), and all were in a high disease activity state [Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2000): 21 points (range: 17-33 points)]. Headache was present in all patients, with some accompanied by dizziness, vomiting, or visual disturbances. Three patients were overweight or obese. Laboratory findings showed anti-dsDNA antibody positivity in 6 patients, anti-ribosomal P protein antibody positivity in 4 patients, and antiphospholipid antibody positivity in 3 patients, with generally decreased complement levels. No specific abnormalities were observed on multimodal imaging. All patients received methylprednisolone pulse therapy (MPPT) combined with immunosuppressive therapy, and 6 patients additionally received biologic agents. Mannitol and other measures were administered to reduce intracranial pressure. The median follow-up duration was 19 months (range: 12-62 months), and all patients achieved remission without recurrence. ② A total of 22 cases (7 cases from this study and 15 cases from the literature) were included in the pooled analysis. Among them, 21 cases (95.5%) were female, with a median age of 13 years. Overweight or obesity was present in 10 cases (45.5%). In 6 patients (27.3%), IH occurred prior to the diagnosis of cSLE. Papilledema was observed in 11 cases (50.0%). Among 15 patients with available disease activity scores, all were in a highly active disease state, with a median SLEDAI-2000 score of 31 points (range: 17-50 points). Antiphospholipid antibodies and anti-ribosomal P protein antibodies were positive in 40.9% and 36.4% of cases, respectively.

cSLE complicated by IH predominantly occurs in adolescent females and is often associated with overweight or obesity and high disease activity. Some patients may present with positive anti-ribosomal P protein and/or antiphospholipid antibodies. IH may precede the diagnosis of cSLE and may occur without papilledema, increasing the risk of missed diagnosis. The presence of marked papilledema should raise concern for potential brain herniation.