原发性中枢神经系统血管炎（primary angiitis of the central nervous system，PACNS）是指主要局限于脑实质、脊髓和软脑膜中小血管的罕见的免疫炎症性疾病，其临床和神经影像学表现多样，已被纳入许多疾病的鉴别诊断中。诊断PACNS并非易事，但是血管造影与脑组织活检的互补有助于诊断PACNS。中国的PACNS病例报道还较少，PACNS的诊断和治疗水平与国外先进水平之间还存在较大差距，因此亟待加强中国的医疗工作者对PACNS的认识。为此，本文围绕PACNS诊断和治疗中国专家共识的内容进行解读，同时结合PACNS 的临床诊治经验，以期提高对PACNS的认识水平。

Primary angiitis of the central nervous system (PACNS) is an uncommon immune-mediated inflammatory disease in which lesions are limited to the small and medium blood vessels of the brain parenchyma, spinal cord and pia mater. Because of its various clinical and neuroimaging manifestations, PACNS has been considered in the differential diagnosis of many diseases. The diagnosis of PACNS is challenging, and the complementation of angiography and brain biopsy is helpful in the diagnosis. Compared with the foreign counterparts, few cases of PACNS had been reported in China, and the level of diagnosis and treatment are far from satisfaction. Therefore, it is urgent to promote the understanding of PACNS among Chinese medical care providers. This paper focuses on the content of Chinese expert consensus on the diagnosis and treatment of PACNS, and combines with the clinical diagnosis and treatment experiences in PACNS, in order to improve the understanding of PACNS.