ArticleScientific reports2022
Primary CNS vasculitis (PCNSV): a cohort study.
Article in Scientific reports, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers, 1 of them a synthesis that pooled it.
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Who cites it
27 citing papers in PubMed, 1 synthesis or guideline pooled it, 36 citations in OpenAlex.
- European Stroke Organisation (ESO) guidelines on Primary Angiitis of the Central Nervous System (PACNS).European stroke journal · 2023Guideline
- Inside the Heterogeneity of Primary CNS Vasculitis: A Single-Center 40-Year Experience.Neurology(R) neuroimmunology & neuroinflammation · 2026Article
- Tumor-mimicking primary CNS vasculitis: illustrative case.Journal of neurosurgery. Case lessons · 2026Article
- Primary central nervous system vasculitis: an update.Journal of neurology · 2026Review
- Central nervous system vasculitis mimicking a brain tumor: A case report.Radiology case reports · 2026Article
- Complete remission of intracranial lymphocytic vasculitis following resection: illustrative case.Journal of neurosurgery. Case lessons · 2026Article
- Differentiating primary angiitis of the central nervous system from paraneoplastic neurological syndrome.BMJ case reports · 2025Article
- Article
- Biopsy-proven PACNS: results from the large, multicentre cohort of cerebral vasculitis patients.Journal of neurology, neurosurgery, and psychiatry · 2025Article
- Biopsy-Confirmed Small Vessel Primary CNS Vasculitis: Clinical Features and Impact of Early Intensive Treatment on Remission.Neurology(R) neuroimmunology & neuroinflammation · 2025Article
- Data-Driven Clustering Approach to Identify Different Phenotypes of Primary Central Nervous System Vasculitis.European journal of neurology · 2025Article
- Case Report: Unilateral relapsing primary central nervous system vasculitis-expanding the phenotype.Frontiers in immunology · 2025Article
- Comparison between primary and secondary central nervous system vasculitis in terms of clinical, biochemical, radiological, histopathological features, and outcomes: a single-center retrospective cohort study.Frontiers in neurology · 2025Article
- Rituximab treatment in Chinese patients with primary angiitis of the central nervous system.Frontiers in neurology · 2025Article
- Rapidly Progressive Primary Central Nervous System Vasculitis: A Rare Case with Multifocal Small and Medium Vessel Involvement.European journal of case reports in internal medicine · 2025Article
- A comparative study of large-vessel and small-vessel primary angiitis of the central nervous system: insights from a Chinese single-center retrospective cohort.Frontiers in immunology · 2025Article
- Article
- Review
- Primary Angiitis of the CNS: Differences in the Profile Between Subtypes and Outcomes From an Indian Cohort.Neurology(R) neuroimmunology & neuroinflammation · 2024Article
- Advancing Knowledge in CNS Vasculitis.Neurology(R) neuroimmunology & neuroinflammation · 2024Article
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Authors and funding
30 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Primary CNS Vasculitis (PCNSV) is a rare inflammatory disorder affecting the blood vessels of the central nervous system. Patients present with a combination of headaches, seizures, and focal neurological deficits. There is usually a diagnostic delay. Treatment is based on observational studies and expert opinion. Our objective was to identify clinical, laboratory, neuroimaging, pathologic or management-related associations with 2 year outcome in patients with primary CNS vasculitis. We conducted a cohort study at a single tertiary care referral centre of prospectively (2018-2019) and retrospectively (2010-2018) identified individuals with primary CNS vasculitis (diagnosis was proven by either brain biopsy or cerebral digital subtraction angiography). Clinical, imaging and histopathologic findings, treatment, and functional outcomes were recorded. Univariate and stepwise multiple logistic regression were applied. P-value<0.05 was considered statistically significant. The main outcome measures were documentation of clinical improvement or worsening (defined by mRS scores) and identification of independent predictors of good functional outcome (mRS 0-2) at 2 years. We enrolled eighty-two biopsy and/or angiographically proven PCNSV cases. The median age at presentation was 34 years with a male predilection and a median diagnostic delay of 23 months. Most patients presented with seizures (70.7%). All patients had haemorrhages on MRI. Histologically lymphocytic subtype was the commonest. Corticosteroids with cyclophosphamide was the commonest medication used. The median mRS at follow-up of 2 years was 2 (0-3), and 65.2% of patients achieved a good functional outcome. Myelitis and longer duration of illness before diagnosis were associated with poorer outcomes. The presence of hemorrhages on SWI sequence of MRI might be a sensitive imaging marker. Treatment with steroids and another immunosuppressant probably reduced relapse rates in our cohort. We have described the third largest PCNSV cohort and multi-centre randomised controlled trials are required to study the relative efficacy of various immunosuppressants.Study registration: CTRI/2018/03/012721.
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