Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Neurodegener Dis. 2014;13(1):38-44. doi: 10.1159/000352020. Epub 2013 Sep 6.
Primary angiitis of the central nervous system (PACNS) is a rare but serious condition. A fraction of patients suffering from PACNS concurrently exhibit pronounced cerebral amyloid angiopathy (CAA) which is characterized by deposits of amyloid-β (Aβ) in and around the walls of small and medium-sized arteries of the brain. PACNS with CAA has been identified as a distinct disease entity, termed Aβ-related angiitis (ABRA). Evidence points to an immune reaction to vessel wall Aβ as the trigger of vasculitis.
To investigate whether the inflammatory response to Aβ has (1) any effect on the status of immune activation in the brain parenchyma and (2) leads to clearance of Aβ from brain parenchyma.
We studied immune activation and Aβ load by quantitative immunohistochemical analysis in brain parenchyma adjacent to affected vessels in 11 ABRA patients and 10 matched CAA controls.
ABRA patients showed significantly increased immune activation and decreased Aβ loads in the brain parenchyma adjacent to affected vessels.
Our results are in line with the hypothesis of ABRA being the result of an excessive immune response to Aβ and show that this can lead to enhanced clearance of Aβ from the brain parenchyma by immune-mediated mechanisms.
原发性中枢神经系统血管炎（PACNS）是一种罕见但严重的疾病。一部分 PACNS 患者同时表现出明显的脑淀粉样血管病（CAA），其特征是大脑中小动脉和中等大小动脉壁内和周围有淀粉样-β（Aβ）沉积。具有 CAA 的 PACNS 已被确定为一种独特的疾病实体，称为 Aβ 相关血管炎（ABRA）。有证据表明，针对血管壁 Aβ 的免疫反应是血管炎的触发因素。
研究 Aβ 的炎症反应是否（1）对脑实质免疫激活状态有任何影响，（2）导致脑实质中 Aβ 的清除。
我们通过对 11 例 ABRA 患者和 10 例匹配的 CAA 对照组受累血管附近脑实质的定量免疫组织化学分析，研究了免疫激活和 Aβ 负荷。
ABRA 患者受累血管附近脑实质的免疫激活明显增加，Aβ 负荷降低。
我们的结果与 ABRA 是 Aβ 过度免疫反应的结果的假设一致，并表明这可以通过免疫介导的机制增强脑实质中 Aβ 的清除。
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