Neurol Neuroimmunol Neuroinflamm. 2026 Jul;13(4):e200573. doi: 10.1212/NXI.0000000000200573. Epub 2026 May 13.
ABSTRACT
BACKGROUND AND OBJECTIVES: Primary CNS vasculitis (PCNSV) is a heterogeneous condition. This study examines a large cohort with long-term follow-up to identify potential disease subsets.
METHODS: We retrospectively analyzed 216 patients with PCNSV (Mayo Clinic, 1983-2023), using standardized diagnostic criteria, classifying by vessel size, histopathology, and outcomes. Subsets and predictors of functional and therapeutic outcomes were evaluated.
RESULTS: Diagnosis was based on cerebral angiography in 142 patients and histologically confirmed in 74. Isolated small vessel involvement was positively associated with mass-lesion presentation (odds ratio [OR] 19.38, p = 0.02), meningeal-enhancing lesions (OR 39.10, p < 0.0001), elevated CSF protein (OR 4.04, p = 0.03), and β-amyloid vascular deposits (OR 23.43, p = 0.0001), but negatively with focal manifestations (OR 0.32, p = 0.04) and cerebral infarcts (OR 0.22, p = 0.003). Lymphocytic vasculitis was linked to younger age at diagnosis (p = 0.006), longer symptom-to-diagnosis interval (p = 0.05), more seizures (p = 0.04), and lower disability (p = 0.003) and mortality (p = 0.008). Necrotizing vasculitis was associated with intracranial hemorrhage (p = 0.008). Two or more relapses occurred in 12.7%, associated with histologic diagnosis (OR 3.15, p = 0.009) and inversely with gadolinium-enhanced lesions (OR 0.33, p = 0.01). Therapy response occurred in 82.9%, long-term remission in 23.6%. Cerebral infarcts, especially multiple, were associated with poor therapy response (OR 0.11, p = 0.03). Histologic diagnosis was inversely associated with long-term remission (OR 0.44, p = 0.03), whereas aspirin use was positively associated (OR 2.8, p = 0.002). A rapidly progressive course occurred in 13.4% of patients and was linked to increasing age (OR 1.34/10 years, p = 0.04), cognitive dysfunction (OR 5.59, p = 0.02), cerebral infarctions (OR 5.02, p = 0.004), and large vessel involvement (OR 3.51, p = 0.02). Gadolinium-enhanced lesions (OR 0.36, p = 0.04) and aspirin (OR 0.42, p = 0.08) were protective. Mortality (21.3%) was associated with older age (HR 1.42, p = 0.002), cognitive dysfunction (HR 3.93, p = 0.006), and cerebral infarctions (HR 1.94, p = 0.03).
DISCUSSION: PCNSV heterogeneity, driven by vessel size and histology, affects presentation and outcomes; our findings offer insights to improve diagnosis and treatment.
PMID:42127334 | DOI:10.1212/NXI.0000000000200573