Department of Neurosurgery, University of Rochester, 601 Elmwood Ave, Rochester, NY, 14642, USA.
Department of Neurologic Surgery, Mayo Clinic, Rochester, MN, USA.
Neurosurg Rev. 2023 Sep 2;46(1):220. doi: 10.1007/s10143-023-02123-z.
Despite more than six decades of extensive research, the etiology of moyamoya disease (MMD) remains unknown. Inflammatory or autoimmune (AI) processes have been suggested to instigate or exacerbate the condition, but the data remains mixed. The objective of the present systematic review was to summarize the available literature investigating the association of MMD and AI conditions as a means of highlighting potential treatment strategies for this subset of moyamoya patients. Using Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, the PubMed, Embase, Scopus, Web of Science, and Cochrane databases were queried to identify studies describing patients with concurrent diagnoses of MMD and AI disease. Data were extracted on patient demographics, clinical outcomes, and treatment. Stable or improved symptoms were considered favorable outcomes, while worsening symptoms and death were considered unfavorable. Quantitative pooled analysis was performed with individual patient-level data. Of 739 unique studies identified, 103 comprising 205 unique patients (80.2% female) were included in the pooled analysis. Most patients (75.8%) identified as Asian/Pacific Islanders, and the most commonly reported AI condition was Graves' disease (57.6%), with 55.9% of these patients presenting in a thyrotoxic state. Of the 148 patients who presented with stroke, 88.5% of cases (n = 131) were ischemic. Outcomes data was available in 152 cases. There were no significant baseline differences between patients treated with supportive therapy alone and those receiving targeted immunosuppressant therapy. Univariable logistic regression showed that surgery plus medical therapy was more likely than medical therapy alone to result in a favorable outcome. On subanalysis of operated patients, 94.1% of patients who underwent combined direct and indirect bypass reported favorable outcomes, relative to 76.2% of patients who underwent indirect bypass and 82% who underwent direct bypass (p < 0.05). On univariable analysis, the presence of multiple AI disorders was associated with worse outcomes relative to having a single AI disorder. Autoimmune diseases have been uncommonly reported in patients with MMD, but the presence of multiple AI comorbidities portends poorer prognosis. The addition of surgical intervention appears to improve outcomes and for patients deemed surgical candidates, combined direct and indirect bypass appears to offer better outcomes that direct or indirect bypass alone.
尽管经过了六十多年的广泛研究，Moyamoya 病（MMD）的病因仍然未知。炎症或自身免疫（AI）过程被认为是引发或加重这种疾病的原因，但数据仍然存在分歧。本系统评价的目的是总结现有的关于 MMD 和 AI 疾病相关性的文献，以突出这组 moyamoya 患者的潜在治疗策略。使用系统评价和荟萃分析的首选报告项目（PRISMA）指南，对 PubMed、Embase、Scopus、Web of Science 和 Cochrane 数据库进行了检索，以确定描述同时患有 MMD 和 AI 疾病的患者的研究。提取患者人口统计学、临床结果和治疗的数据。稳定或改善的症状被认为是有利的结果，而症状恶化和死亡被认为是不利的结果。对个体患者水平的数据进行了定量汇总分析。在 739 项独特的研究中，有 103 项研究包括 205 名独特的患者（80.2%为女性）被纳入汇总分析。大多数患者（75.8%）被确定为亚洲/太平洋岛民，最常见的 AI 疾病是格雷夫斯病（57.6%），其中 55.9%的患者处于甲状腺功能亢进状态。在 148 名出现中风的患者中，88.5%（n=131）为缺血性中风。152 例中有结局数据。接受单纯支持治疗的患者与接受靶向免疫抑制治疗的患者之间没有显著的基线差异。单变量逻辑回归显示，手术联合药物治疗比单纯药物治疗更有可能产生有利的结果。对手术患者的亚分析显示，接受直接和间接旁路联合手术的患者中，94.1%报告了有利的结果，而间接旁路手术患者的比例为 76.2%，直接旁路手术患者的比例为 82%（p<0.05）。单变量分析显示，存在多种 AI 疾病与单一 AI 疾病相比，预后更差。MMD 患者中罕见报道自身免疫性疾病，但存在多种 AI 合并症预示着预后更差。手术干预的加入似乎改善了结果，对于被认为是手术候选者的患者，直接和间接旁路联合手术似乎比单独直接或间接旁路手术提供更好的结果。
