12例GFAP-IgG阳性患儿重叠综合征的临床特征分析

The Analysis of Clinical Characteristics of Overlapping Syndrome in 12 Children with GFAP-IgG Positive

摘要: 背景：自身免疫性胶质纤维酸性蛋白(GFAP)星形细胞病变是一种自身免疫性中枢神经系统疾病，GFAP-IgG是其特异性抗体，现报道大约有30%~40%患者常与其他抗体共存。目的：探讨儿童自身免疫性GFAP星形细胞病变的重叠抗体临床特征分析。方法：收集2020年1月~2022年12月在重庆医科大学儿童医院住院治疗的43例脑脊液和/或血清GFAP抗体阳性的中枢神经系统疾病患者，回顾性分析其中出现重叠综合征的12例患儿的临床资料。结果：共纳入GFAP-IgG阳性患者43例，其中重叠综合征12例。其中AQP4抗体阳性3例，MOG抗体阳性2例，NMDAR抗体及MOG抗体同时阳性3例，NMDAR抗体阳性1例；抗Sulfatide抗体IgG阳性2例，抗GM1、抗GD1b抗体IgM阳性1例。我们发现重叠抗体患儿有近50%出现复发，尤其以重叠NMDAR抗体和AQP4抗体突出，需联合吗替麦考酚酯等免疫抑制剂治疗。

Abstract: Background: Autoimmune glial fibrillary acidic protein astrocytopathy is an autoimmune central system disease, and GFAP-IgG is specific. It is reported that about 30% to 40% of patients often co-exist with other antibodies. Objective: To investigate the clinical characteristics of overlapping an-tibodies in autoimmune GFAP astrocytopathyin children. Methods: We collected 43 patients with positive GFAP-IgG in cerebrospinal fluid and/or serum, and treated in Children’s Hospital of Chong-qing Medical University from January 2020 to December 2022, and analyzed the clinical data of 12 patients with overlapping syndrome retrospectively. Results: 43 patients with positive GFAP-IgG which were included, of whom 12 were defined as overlapping syndrome. Among them, 3 cases were positive for AQP4 antibody, 2 cases for MOG antibody, 3 cases for both NMDAR antibody and MOG antibody, 1 case for NMDAR antibody, 2 cases for anti-Sulfatide-IgG, and 1 case for anti-GM1 antibody and anti-GD1b antibody. We found that about 50% of pediatric patients with overlapping antibodies relapsed, especially with overlapping NMDAR antibody and AQP4 antibody, which needed to be treated with immunosuppressants such as mycophenolate mofetil.