Department of Rheumatology, Medical University of Lodz, Łódź, Poland.
Department of Immunology and Allergy, Medical University of Lodz, Łódź, Poland.
Rheumatol Int. 2024 Feb;44(2):211-222. doi: 10.1007/s00296-023-05461-9. Epub 2023 Sep 30.
Vasculitis of the central nervous system can be a localized process, such as primary angiitis of the central nervous system (PACNS), or systemic vasculitis, such as ANCA-associated vasculitis (AAV). Since both conditions share neurological manifestations, the following review will discuss the neurological aspects of both. This review aims to provide a comprehensive comparison of the pathogenesis, clinical manifestation and assessment, diagnostic workup, and treatment protocol for both PACNS and AAV with central nervous system involvement. To provide a comprehensive comparison and update, a literature review was conducted using PubMed and Ovid databases (Embase and Medline). Then, the references were retrieved, screened, and selected according to the inclusion and exclusion criteria. PACNS and AAV share similarities in clinical presentation and neurological symptoms, especially in terms of headache, focal deficits, and cognitive impairment. Additionally, both conditions may exhibit similarities in laboratory and radiological findings, making brain biopsy the gold standard for differentiation between the two conditions. Moreover, the treatment protocols for PACNS and AAV are nearly identical. Comparing PACNS and AAV with CNS involvement highlights the similarities in clinical presentation, radiological findings, and treatment protocols between the two conditions. Further research should focus on establishing a practical diagnostic protocol.
中枢神经系统血管炎可以是局灶性过程，如原发性中枢神经系统血管炎（PACNS），或系统性血管炎，如 ANCA 相关性血管炎（AAV）。由于这两种疾病都有神经表现，因此以下综述将讨论这两种疾病的神经方面。本综述旨在提供 PACNS 和伴有中枢神经系统受累的 AAV 的发病机制、临床表现和评估、诊断检查和治疗方案的全面比较。为了进行全面比较和更新，使用 PubMed 和 Ovid 数据库（Embase 和 Medline）进行了文献回顾。然后，根据纳入和排除标准检索、筛选和选择参考文献。PACNS 和 AAV 在临床表现和神经症状方面存在相似之处，尤其是头痛、局灶性缺损和认知障碍方面。此外，这两种疾病在实验室和影像学发现方面也可能存在相似之处，因此脑活检是区分这两种疾病的金标准。此外，PACNS 和 AAV 的治疗方案几乎相同。比较伴有中枢神经系统受累的 PACNS 和 AAV 突出了这两种疾病在临床表现、影像学发现和治疗方案方面的相似性。进一步的研究应侧重于建立实用的诊断方案。
