原发性中枢神经系统血管炎（primary angiitis of the central nervous system，PACNS）是累及大脑和脊髓的罕见的血管炎症性疾病。目前，PACNS 的诊断主要依赖于临床症状和体征、影像学表现、脑脊液检查结果以及脑组织活检结果。磁共振血管成像（magnetic resonance angiography，MRA）和数字减影血管造影（digital subtraction angiography，DSA）可用于评估PACNS 的血管病变，而通过脑组织活检可以明确病理诊断。回顾性研究显示，环磷酰胺联合糖皮质激素可作为PACNS 诱导期的治疗方案；维持期的治疗，可采用免疫抑制剂如硫唑嘌呤等。此外，抗CD20 单克隆抗体利妥昔单抗等生物制剂的治疗获益亦有所报道。然而，鉴于PACNS 的罕见性和异质性，目前尚缺乏与诊断和治疗策略相关的确切的临床证据，因此PACNS 的诊断和治疗在临床实践中依然极具挑战性。今后，有待进一步探索PACNS 的发病机制，以期为早期诊断和提高治疗效果提供科学依据。

The primary angiitis of the central nervous system (PACNS) is a rare vascular inflammatory disease that involving the brain and spinal cord. Currently, the diagnosis of PACNS is mainly dependent on clinical manifestations and signs, imaging, cerebrospinal fluid examination and brain biopsy. The magnetic resonance angiography (MRA) and digital subtraction angiography (DSA) are employed in PACNS to assess the vascular lesions. Pathological diagnosis can be confirmed by brain biopsy. Retrospective studies have shown that cyclophosphamide combined with glucocorticoids can be used as a therapeutic regimen in the induction phase of PACNS, while immunosuppressive agents such as azathioprine can be used as maintaining therapy. In addition, the therapeutic benefits of biological agents such as rituximab, an anti-CD20 monoclonal antibody, have also been reported. However, due to the rarity and heterogeneity of PACNS, there is no definite clinical evidence related to diagnosis and treatment strategies, so the diagnosis and treatment of PACNS is still very challenging in clinical practice. Therefore, It is urgent to explore the pathogenesis of PACNS in order to provide scientific basis for early diagnosis and improvement of therapeutic effect.