Yang Jie, Jiang Lihong, Yao Haiyan, Huang Li, Long Youming
Department of Neurology, The Second Affiliated Hospital of GuangZhou Medical University, Guangzhou, Guangdong Province, People's Republic of China.
Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and The Ministry of Education of China, Institute of Neuroscience and the Second Affiliated Hospital of GuangZhou Medical University, Guangzhou, Guangdong Province, People's Republic of China.
Neuropsychiatr Dis Treat. 2022 Jun 1;18:1099-1105. doi: 10.2147/NDT.S364246. eCollection 2022.
Currently, no uniform diagnostic criteria or treatment consensus is available for patients with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A). The aim of this registry is to develop diagnostic and therapeutic recommendations for GFAP-A based on clinical features, neuroimaging, neuroelectrophysiological examinations, laboratory tests, specific antibody tests, immunotherapy, and prognosis.
This multicenter, nationwide ambispective registry includes twenty-seven hospitals in China. From January 2020 to December 2022, consecutive hospitalized patients with symptoms of meningoencephalitis, as well as GFAP-IgG positive cerebrospinal fluid (CSF) or serum will be invited to join this study. It is conservatively estimated that over 300 patients will join the study. Data on demographics, medical history, treatment details and imaging features will be collected after discharge. Outcome events of interest will include modified Rankin Scale (mRS) and Expanded Disability Status Scale (EDSS), readmission with relapsed meningoencephalomyelitis, all-cause mortality, and mortality resulting from complications of GFAP-A. The follow-up will be conducted at six months and twelve months after discharge. Univariate and multivariate regression models will be used to calculate identify independent predictors of outcomes. Stratification analysis will be used to test whether results are similar between key subgroups.
This study will describe the risk factors, disease course, response to immunotherapy, and long-term prognosis of a large cohort of GFAP-A patients. By using these data, a relatively rational recommendation process for the diagnosis and treatment of GFAP-A will be developed.
ChiCTR2000041291.
目前，自身免疫性胶质纤维酸性蛋白星形细胞病（GFAP-A）患者尚无统一的诊断标准或治疗共识。本登记研究的目的是基于临床特征、神经影像学、神经电生理检查、实验室检查、特异性抗体检测、免疫治疗及预后情况，制定GFAP-A的诊断和治疗建议。
这项多中心、全国性的前瞻性登记研究纳入了中国的27家医院。2020年1月至2022年12月期间，连续收治的有脑膜脑炎症状且脑脊液（CSF）或血清GFAP-IgG阳性的住院患者将被邀请加入本研究。据保守估计，超过300名患者将参与该研究。出院后将收集人口统计学、病史、治疗细节及影像学特征等数据。感兴趣的结局事件将包括改良Rankin量表（mRS）和扩展残疾状态量表（EDSS）、复发性脑膜脑脊髓炎再次入院、全因死亡率以及GFAP-A并发症导致的死亡率。随访将在出院后6个月和12个月进行。将使用单因素和多因素回归模型来计算并确定结局的独立预测因素。将采用分层分析来检验关键亚组之间的结果是否相似。
本研究将描述一大群GFAP-A患者的危险因素、病程、免疫治疗反应及长期预后。通过使用这些数据，将制定一个相对合理的GFAP-A诊断和治疗推荐流程。
ChiCTR2000041291。
