ORIGINAL RESEARCH article
Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders: Autoinflammatory Disorders
A Comparative Study of Large-Vessel and Small-Vessel Primary Angiitis of the Central Nervous System (PACNS): Insights from a Chinese Single-Center Retrospective Cohort
Provisionally accepted
- 1People's Hospital of Zhengzhou University, Zhengzhou, China
- 2Henan Provincial People's Hospital, Zhengzhou, China
- 3People's Hospital of Henan University, Zhengzhpu, China
- 4People's Hospital of Henan University, Zhengzhou, China
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BACKGROUND: Primary angiitis of the central nervous system (PACNS) is a rare immune-mediated vasculitis with distinct subtypes (large/medium-vessel [LV-PACNS] and small-vessel [SV-PACNS]). This study aims to provide evidence-based data from the Chinese population for improving the subtype-specific management system of PACNS. METHODS: This retrospective single-center study enrolled 47 PACNS patients (29 with SV-PACNS, 18 with LV-PACNS) who met the 1988 Calabrese and Mallek diagnostic criteria, with a follow-up duration ≥1 year. Clinical phenotypes, imaging characteristics, treatment, prognosis, and factors influencing prognosis were retrospectively analyzed in both groups. RESULTS: Compared with LV-PACNS, SV-PACNS had more severe initial neurological impairment (baseline modified Rankin Scale mRS score: 3 vs. 2, p=0.043) and a longer median time from onset to treatment initiation (154 days vs. 58.5 days, p=0.013). In terms of clinical features, LV-PACNS had a higher incidence of cerebrovascular events (88.9% vs. 58.2%, p=0.027) and limb weakness/sensory abnormalities (83.3% vs. 34.5%, p=0.001), while SV-PACNS had a higher incidence of tumor-like lesions (41.4% vs. 5.6%, p=0.008). Imaging features showed that 100% of LV-PACNS patients had cerebrovascular stenosis, of which 94.4% exhibited circumferential vascular wall enhancement; and compared with SV-PACNS, they had a higher incidence of ischemic infarction (66.7% vs 20.7%, p=0.002). Multivariate analysis confirmed that time from onset to treatment was an independent risk factor for poor 1-year prognosis (mRS score > 2) in both subtypes (SV-PACNS: OR=1.012, p=0.021; LV-PACNS: OR=1.048, p=0.040). CORRESPONDENCE:Weizhou Zang; tiger_zang@163.com †These authors have contributed equally to this work CONCLUSIONS: This study identified the core clinical and neuroimaging differences between LV-PACNS and SV-PACNS, and pointed out that treatment delay are the main issues affecting prognosis.
Keywords: Primary central nervous system vasculitis, Clinical features, Imaging features, prognosis, CNS immune diseases
Received: 14 Oct 2025; Accepted: 24 Nov 2025.
Copyright: © 2025 Wang, Lu, Li, Zhao, He, Xiang and Zang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Weizhou Zang
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