Institute of Neuroscience and Department of Neurology, Second Affiliated Hospital of Guangzhou Medical University, Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China, Guangzhou, China.
Guangdong Province Key Laboratory of Brain Function and Disease, Guangzhou, China.
Eur J Neurol. 2021 Jun;28(6):2121-2125. doi: 10.1111/ene.14778. Epub 2021 Mar 6.
We aimed to report the pathological features of T lymphocytes in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A).
A retrospective pathological analysis of patients with GFAP-A was performed.
Eight patients with GFAP-immunoglobulin G (IgG) and pathological data were included. Their biopsy findings were similar, and all showed marked lymphocytic infiltration in the white matter, with perivascular predominance. The lymphocytic infiltration was predominantly composed of CD8 T lymphocytes rather than CD4 T lymphocytes, except in one patient who had overlapping positive myelin oligodendrocyte glycoprotein-IgG. Unlike CD4 T cells, CD8 T cells were frequently observed adjacent to dystrophic neurons and astrocytes. There was also diffuse infiltration by CD68 and CD163 macrophages. CD8 astrocytes were identified in two samples, but no CD4 astrocytes were observed.
A predominance of CD8 T cells may be an important pathological and diagnostic feature in GFAP-A.
我们旨在报告自身免疫性胶质纤维酸性蛋白(GFAP)星形胶质细胞病(GFAP-A)中 T 淋巴细胞的病理学特征。
对 GFAP-A 患者进行回顾性病理分析。
纳入 8 例具有 GFAP-IgG 和病理资料的患者。他们的活检结果相似,均显示白质中有明显的淋巴细胞浸润,以血管周围浸润为主。淋巴细胞浸润主要由 CD8 T 淋巴细胞组成,而非 CD4 T 淋巴细胞,仅 1 例患者存在重叠性髓鞘少突胶质细胞糖蛋白-IgG 阳性。与 CD4 T 细胞不同,CD8 T 细胞常与退行性神经元和星形胶质细胞相邻。还可见 CD68 和 CD163 巨噬细胞弥漫浸润。在两个样本中鉴定出 CD8 星形胶质细胞,但未观察到 CD4 星形胶质细胞。
CD8 T 细胞为主可能是 GFAP-A 的重要病理和诊断特征。