1. Disease Information

1.1 What is the disease? (concise overview)

Primary angiitis of the CNS (PACNS/PCNSV) is a rare inflammatory disorder of CNS blood vessels that is restricted to the brain/spinal cord/meninges (i.e., no systemic vasculitis) and presents with heterogeneous neurologic syndromes such as headache, encephalopathy, stroke syndromes, and seizures. (pascarella2023europeanstrokeorganisation pages 1-2, sheikh2024primarycnsvasculitis pages 1-2, hoshina2024vasculitisinthe pages 1-2)

Authoritative definition/clinical framing (ESO guideline, Oct 2023): PACNS is treated as an isolated CNS vasculitis with very low-quality evidence across many diagnostic/treatment questions; histopathology is emphasized as the diagnostic reference standard and there is no single specific neuroimaging pattern. (pascarella2023europeanstrokeorganisation pages 1-2)

1.2 Key identifiers and codes

Only some coding/identifier systems were explicitly present in the retrieved sources:

• ICD-9: 437.4 (reported as used for case identification in a CNS vasculitis cohort). (hoshina2024vasculitisinthe pages 2-3)
• ICD-10: I67.7 “Cerebral arteritis, not elsewhere classified” (reported as used for case identification). (hoshina2024vasculitisinthe pages 2-3)

Not found in retrieved evidence: MONDO ID, MeSH ID, Orphanet ID, OMIM ID, ICD-11 code. (pascarella2023europeanstrokeorganisation pages 1-2, hoshina2024vasculitisinthe pages 2-3)

1.3 Synonyms / alternative names

Common synonyms used in the recent literature include: - PACNS = “Primary angiitis of the central nervous system” (ESO guideline terminology). (pascarella2023europeanstrokeorganisation pages 1-2) - PCNSV/PCNSV = “Primary CNS vasculitis” (used interchangeably in 2024 clinical/imaging series). (sheikh2024primarycnsvasculitis pages 1-2, d’aniello2024thecontributionof pages 1-2) - Subclassification terms (especially in guidelines): LV-PACNS (large/medium vessel predominant) and SV-PACNS (small-vessel predominant). (pascarella2023europeanstrokeorganisation pages 1-2)

1.4 Evidence source type (patient vs aggregated)

The retrieved evidence is primarily aggregated disease-level resources (ESO guideline) plus retrospective single-center cohorts/series and imaging-focused case series (human clinical). (pascarella2023europeanstrokeorganisation pages 1-2, hoshina2024vasculitisinthe pages 2-3, d’aniello2024thecontributionof pages 1-2, agarwal2024theroleof pages 11-12)

Identifier/synonym summary table

Table: This table compiles the disease names, abbreviations, coding systems, and terminology explicitly documented in the retrieved evidence for CNS vasculitis and PACNS/PCNSV. It is useful for normalizing nomenclature and identifying which ontology identifiers were or were not available from the current evidence set.

2. Etiology

2.1 Disease causal factors

• Primary CNS vasculitis (PACNS/PCNSV): by definition, vasculitic inflammation restricted to CNS vessels without an identified systemic cause. (scoppettuolo2025comparisonbetweenprimary pages 1-2, hoshina2024vasculitisinthe pages 1-2)
• Secondary CNS vasculitis (SCNSV/SACNS): inflammation of CNS vessels secondary to another condition, including infection and connective tissue disease (CTD)-associated causes in 2024 cohort data. (hoshina2024vasculitisinthe pages 2-3, scoppettuolo2025comparisonbetweenprimary pages 1-2)

Large single-center cohort etiology breakdown (University of Utah, 2011–2022; n=44): - Primary CNS vasculitis overall: 56.8% - PACNS: 17/44 (38.6%) - Inflammatory cerebral amyloid angiopathy (CAA; includes ABRA): 8/44 (18.2%) - Secondary CNS vasculitis: 43.2% - Infectious: 10/44 (22.7%) - CTD-associated: 6/44 (13.6%) - Systemic vasculitis: 3/44 (6.8%) (hoshina2024vasculitisinthe pages 1-2)

2.2 Risk factors

The retrieved sources emphasize diagnostic differentiation rather than enumerating prospective risk factors. However, secondary disease signals are reported: - Fever occurred more often in secondary than primary CNS vasculitis (0% vs 26.3%; p=0.01), supporting infection/CTD suspicion when systemic features are present. (hoshina2024vasculitisinthe pages 2-3)

2.3 Protective factors / gene–environment interactions

Not identified in retrieved sources.

3. Phenotypes

3.1 Core clinical phenotypes (adult cohorts/series)

Across the recent adult cohort/series literature: - In a 44-patient CNS vasculitis cohort, common presenting features were headache (63.6%), altered cognition (43.2%), and focal weakness (38.6%). (hoshina2024vasculitisinthe pages 2-3) - A 2024 PCNSV single-center series emphasizes heterogeneous presentations including headache, seizures, cognitive impairment, and delayed diagnosis (median symptom duration 36 weeks before diagnosis in that series). (sheikh2024primarycnsvasculitis pages 1-2)

3.2 Imaging-linked phenotypes (high-yield patterns in 2024 imaging studies)

• Biopsy-proven PCNSV (n=56): SWI hemorrhages in 96.4% (54/56) and parenchymal enhancement in 96.3% (52/54), with dot-linear enhancement being the most prevalent enhancement pattern (87%). (agarwal2024theroleof pages 11-12)
• HR vessel-wall imaging cohort (n=14) included mostly stroke-like presentations (78.7% stroke-like episodes). (d’aniello2024thecontributionof pages 2-4)

3.3 Quality-of-life / disability signals

In the 2024 PCNSV series (n=5), most patients required walking aids initially and some required them at follow-up, illustrating persistent disability in a subset despite immunotherapy. (sheikh2024primarycnsvasculitis pages 1-2)

3.4 Suggested HPO terms

A phenotype-to-HPO mapping table (with frequencies where available from recent cohorts) is provided below.

Table: This table summarizes reported clinical phenotypes and suggested HPO mappings for CNS vasculitis/PACNS, emphasizing frequencies and distinguishing features from recent cohorts and imaging studies. It is useful for knowledge-base phenotype annotation and for separating primary from secondary CNS vasculitis.

4. Genetic / Molecular Information

4.1 Causal genes / pathogenic variants

No PACNS/PCNSV causal genes or pathogenic variants were explicitly reported in the retrieved 2023–2024 core sources.

4.2 Molecular/histologic signatures (cellular composition)

In pediatric small-vessel CNS vasculitis biopsy material, immune infiltrates included CD3, CD4, CD8, and CD20 cells as predominant components (T-cell and B-cell rich angiocentric inflammation). (d’aniello2024thecontributionof pages 2-4)

5. Environmental Information

Not systematically addressed in retrieved 2023–2024 sources; secondary/infectious etiologies are recognized as major environmental/exogenous contributors in “secondary CNS vasculitis” categories. (hoshina2024vasculitisinthe pages 2-3)

6. Mechanism / Pathophysiology

6.1 Current understanding (causal chain)

A consistent mechanistic model across guideline/cohort/imaging evidence is: 1. Immune-mediated inflammation targets CNS vessel walls (small, medium, and/or large vessels), often with transmural involvement (histopathology emphasized in the ESO guideline). (pascarella2023europeanstrokeorganisation pages 1-2) 2. Vessel wall inflammation leads to luminal compromise, endothelial activation, and thrombosis/occlusion, producing ischemic injury; small-vessel fragility and inflammation also contribute to microhemorrhages detectable on SWI. (sheikh2024primarycnsvasculitis pages 1-2, agarwal2024theroleof pages 11-12) 3. Parenchymal injury manifests as multifocal neurologic deficits (headache/encephalopathy/focal deficits/seizures) and MRI abnormalities (white-matter lesions, diffusion restriction, enhancement). (hoshina2024vasculitisinthe pages 2-3, sheikh2024primarycnsvasculitis pages 1-2, d’aniello2024thecontributionof pages 1-2)

6.2 Histopathologic patterns

In a recent CNS vasculitis cohort, histologic phenotypes included lymphocytic, granulomatous, and necrotizing patterns. (hoshina2024vasculitisinthe pages 2-3)

6.3 Pathophysiology-related imaging biomarkers (2024)

• Vessel-wall MRI: CNS vasculitis frequently shows intense, concentric vessel-wall enhancement (13/14 had enhancement; 84.6% intense; 92.3% concentric). (d’aniello2024thecontributionof pages 1-2)
• DWI correlation: DWI alterations corresponded with vessel-wall enhancement territories in 77%, consistent with ischemic/hypoperfusion injury downstream of inflamed vessels. (d’aniello2024thecontributionof pages 1-2)
• SWI microhemorrhages: extremely common in biopsy-proven PCNSV (96.4%). (agarwal2024theroleof pages 11-12)

6.4 Suggested ontology terms (examples)

Because formal ontology IDs were not provided in the sources, the following are suggested (not evidence-derived IDs): - GO biological processes (suggested): leukocyte migration; T cell activation; B cell activation; cytokine-mediated signaling; vasculature development/inflammation; endothelial cell activation. - CL cell types (suggested): T cell; CD4-positive T cell; CD8-positive T cell; B cell; endothelial cell.

7. Anatomical Structures Affected

7.1 Organ/system level

Primary disease is confined to the central nervous system: vessels of the brain, spinal cord, and meninges/leptomeninges. (sheikh2024primarycnsvasculitis pages 1-2, d’aniello2024thecontributionof pages 2-4)

7.2 Localization patterns (imaging)

• Vessel-wall MRI cohort: enhancement affected the anterior circulation in 69.2% of patients and posterior circulation in 15.4%. (d’aniello2024thecontributionof pages 1-2)
• Biopsy-proven PCNSV imaging series: frontal lobe lesions predominated; inferior cerebellar lesions were also frequent; lesions often bilateral and diffuse within white matter. (agarwal2024theroleof pages 11-12)

8. Temporal Development

8.1 Onset and diagnostic delay

PACNS/PCNSV can have subacute-to-chronic evolution and diagnostic delay. In a 2024 PCNSV series, symptoms persisted a median 36 weeks before diagnosis (range 3 weeks to 4 years). (sheikh2024primarycnsvasculitis pages 1-2)

8.2 Course patterns

Relapsing disease is recognized; in a biopsy-confirmed small-vessel PCNSV cohort, overall relapse rate was 19% (2025 evidence, included here due to limited relapse statistics in 2023–2024 sources). (kharal2024predictivevalueof pages 1-2)

9. Inheritance and Population

9.1 Epidemiology

Quantitative incidence was not systematically reported in the 2023 ESO guideline abstract, but a recent cohort-comparison paper cites an estimated incidence of ~2.4 cases per million person-years for PACNS (not a 2023–2024 primary epidemiology study; included as a commonly-cited estimate). (scoppettuolo2025comparisonbetweenprimary pages 1-2)

A 2024 single-center cohort provides internal proportions of primary vs secondary etiologies but is not population-based. (hoshina2024vasculitisinthe pages 2-3)

9.2 Demographics

• Utah cohort: median age 54; 25% male overall. (hoshina2024vasculitisinthe pages 2-3)
• PCNSV series (n=5): median onset age 31; 80% male (small series; likely not generalizable). (sheikh2024primarycnsvasculitis pages 1-2)

No genetic inheritance pattern is established for PACNS in the retrieved evidence.

10. Diagnostics

10.1 Diagnostic criteria / expert guidance

ESO 2023 key expert statements (abstract-level): - Overall evidence quality is very low across many diagnostic/treatment questions. - CSF pleocytosis/hyperproteinorrachia lack evidence as diagnostic tools. - Non-invasive vascular imaging must be interpreted with caution (lack of validation vs DSA/histopathology). - No neuroimaging pattern is specific for PACNS. - Multidisciplinary expert-center approach is recommended. (pascarella2023europeanstrokeorganisation pages 1-2)

10.2 Imaging advances and diagnostic performance signals (2024)

Key 2024 developments emphasize high-resolution vessel-wall MRI and advanced MRI signatures: - HR vessel-wall MRI frequently shows intense, concentric enhancement, supporting inflammatory vasculopathy evaluation. (d’aniello2024thecontributionof pages 1-2) - In biopsy-proven PCNSV, SWI microhemorrhages and dot-linear enhancement patterns were proposed as minor diagnostic criteria in a large single-center series (56 biopsy-positive). (agarwal2024theroleof pages 11-12) - Quantitative VWMRI metrics combined with clinical syndrome and CSF WBC can help discriminate PACNS from intracranial atherosclerotic disease: e.g., C/E ratio >1 (OR 115), concentricity ≥50% (OR 55), irregularity <50% (OR 55), seizure (OR 23), encephalopathy (OR 7.6). (kharal2024predictivevalueof pages 1-2)

10.3 Biopsy yield in real-world cohorts

• Utah cohort: biopsy confirmed vasculitis in 7/10 biopsied PACNS cases and 4/4 inflammatory CAA cases. (hoshina2024vasculitisinthe pages 2-3)
• Biopsy-proven PCNSV imaging series: 80 biopsies performed, 56 positive (70%). (agarwal2024theroleof pages 11-12)

Diagnostic modalities table

Table: This table summarizes the main 2023-2024 diagnostic tests used in CNS vasculitis/PACNS and the most relevant quantitative findings from recent guideline, cohort, and imaging studies. It is useful for comparing what each modality measures, how strongly it supports diagnosis, and where its limitations lie.

11. Outcome / Prognosis

11.1 Mortality

In a 2011–2022 CNS vasculitis single-center cohort (n=44), overall mortality was 20.5% (primary 16.0%, secondary 26.3%). (hoshina2024vasculitisinthe pages 2-3)

11.2 Functional outcomes

In the 2024 PCNSV series with median 56-month follow-up (n=5), outcomes varied: some improved, some remained stable, and one deteriorated; mobility aids remained necessary for some patients after treatment. (sheikh2024primarycnsvasculitis pages 1-2)

Robust long-term survival or disability statistics (5-year/10-year) were not available in the retrieved 2023–2024 evidence.

12. Treatment

12.1 Current standard practice (induction/maintenance)

Across guideline and contemporary clinical series, common treatment paradigms include: - Induction: high-dose corticosteroids, often with cyclophosphamide for severe disease. (sheikh2024primarycnsvasculitis pages 1-2, rice2026primarycentralnervous pages 2-4) - Maintenance / steroid-sparing: azathioprine, methotrexate, or mycophenolate mofetil; rituximab is used in some cohorts as maintenance or for refractory disease. (sheikh2024primarycnsvasculitis pages 1-2, hoshina2024vasculitisinthe pages 1-2, rice2026primarycentralnervous pages 2-4)

ESO 2023 perspective: treatment recommendations for induction and maintenance reflect substantial uncertainty because controlled trial evidence is sparse. (pascarella2023europeanstrokeorganisation pages 1-2)

12.2 Real-world implementation statistics

In the Utah cohort (n=44): - IV methylprednisolone was predominant induction therapy (63.6%). - Cyclophosphamide was the most used adjunctive therapy. - Maintenance therapy included cyclophosphamide, rituximab, azathioprine, and mycophenolate (often with prednisone). (hoshina2024vasculitisinthe pages 2-3, hoshina2024vasculitisinthe pages 1-2)

12.3 Suggested MAXO terms (examples)

Suggested (not source-derived IDs): systemic glucocorticoid therapy; cyclophosphamide therapy; B-cell depletion therapy (rituximab); immunosuppressive maintenance therapy; plasma exchange; brain biopsy; MRI; cerebral angiography.

Treatment summary table

Table: This table summarizes induction, maintenance, and adjunctive treatment strategies for CNS vasculitis/PACNS and the main quantitative outcomes available from the retrieved evidence. It is useful for distinguishing consensus-based practice from the few cohorts that report remission, relapse, and mortality statistics.

13. Prevention

No primary prevention strategy is established for PACNS/PCNSV in the retrieved evidence; prevention is largely secondary/tertiary, focused on early recognition, exclusion of mimics/secondary causes, and relapse prevention with maintenance immunosuppression (expert practice). (pascarella2023europeanstrokeorganisation pages 1-2, rice2026primarycentralnervous pages 2-4)

14. Other Species / Natural Disease

No animal/natural disease information was identified in the retrieved sources.

15. Model Organisms

No model organism systems were described in the retrieved sources.

Recent developments (2023–2024 highlights)

1. First comprehensive European PACNS guideline using GRADE (Oct 2023): emphasizes very low evidence quality, non-specific MRI patterns, and the need for expert-center multidisciplinary care and standardized neuroimaging/reporting. (pascarella2023europeanstrokeorganisation pages 1-2)
2. Rapid growth in vessel-wall MRI evidence (Apr 2024): intense, concentric enhancement frequently observed in CNS vasculitis, supporting VWI as an adjunct to conventional MRI/MRA/DSA. (d’aniello2024thecontributionof pages 1-2)
3. Advanced MRI signatures in biopsy-proven PCNSV (Feb 2024): near-universal SWI hemorrhages and high rates of contrast enhancement with characteristic patterns; proposal to incorporate SWI hemorrhage and dot-linear enhancement as minor diagnostic criteria. (agarwal2024theroleof pages 11-12)
4. Quantitative VWMRI + CSF/clinical predictors (Aug 2024): strong ORs for VWMRI geometry metrics and clinical syndromes distinguishing PACNS from intracranial atherosclerosis in a stroke/vasculopathy cohort. (kharal2024predictivevalueof pages 1-2)

Clinical trials and translational pipeline (real-world implementations)

• NCT03166319 (Mayo Clinic; completed; start 2017-07-24, completion 2020-11-24): pilot interventional diagnostic study of intracranial HR vessel-wall MRI for suspected PACNS; enrollment 15; repeated MRI/MRA/VWI with gadolinium; imaging read by two neuroradiologists. URL: https://clinicaltrials.gov/study/NCT03166319 (NCT03166319 chunk 1)
• NCT05168475 BIOVAS (Cambridge University Hospitals NHS Foundation Trust; Phase 2; terminated due to funding withdrawal): randomized double-blind placebo-controlled modified-crossover trial planning sequential exposure to infliximab, rituximab, tocilizumab and placebo in refractory primary non-ANCA-associated vasculitis including PACNS; enrollment 22; primary endpoint treatment failure up to 720 days defined via BVAS/PVAS modifications. URL: https://clinicaltrials.gov/study/NCT05168475 (NCT05168475 chunk 1)
• NCT00659776 (OHSU; Phase 2; terminated): ferumoxytol MRI for CNS inflammation/vascular processes; enrollment 255; diagnostic imaging endpoints and histologic/EM correlation. URL: https://clinicaltrials.gov/study/NCT00659776 (NCT00659776 chunk 1)

Evidence limitations and gaps (explicit)

• Ontology identifiers (MONDO/MeSH/Orphanet/OMIM/ICD-11) were not present in the retrieved evidence; they should be added by direct ontology lookup in dedicated resources (e.g., MONDO/Orphanet/MeSH browsers) rather than inferred. (hoshina2024vasculitisinthe pages 2-3)
• Many “classic” PACNS statements rely on older cohorts and expert consensus; the ESO guideline explicitly characterizes the evidence base as very low quality for many clinical questions, reinforcing that 2023–2024 advances are primarily in imaging characterization rather than randomized treatment trials. (pascarella2023europeanstrokeorganisation pages 1-2)
• PMIDs were not provided in the retrieved full-text evidence snippets; the report therefore cites DOI/URLs and journal metadata from the retrieved sources.

Key cited sources (2023–2024 prioritized)

• Pascarella R, et al. European Stroke Organisation (ESO) guidelines on PACNS. European Stroke Journal. Oct 2023. https://doi.org/10.1177/23969873231190431 (pascarella2023europeanstrokeorganisation pages 1-2)
• D’Aniello S, et al. Vessel wall MRI in primary/secondary CNS vasculitis. Diagnostics. Apr 2024. https://doi.org/10.3390/diagnostics14090927 (d’aniello2024thecontributionof pages 1-2)
• Agarwal S, et al. SWI and contrast MRI in biopsy-proven PCNSV (case series). Scientific Reports. Feb 2024. https://doi.org/10.1038/s41598-024-55222-2 (agarwal2024theroleof pages 11-12)
• Kharal MMGA, et al. Clinical/CSF/VWMRI predictors of PACNS vs ICAD. Neurology Clinical Practice. Aug 2024. https://doi.org/10.1212/cpj.0000000000200321 (kharal2024predictivevalueof pages 1-2)
• Hoshina Y, et al. Etiology and outcomes in CNS vasculitis cohort (n=44). The Neurohospitalist. Dec 2024. https://doi.org/10.1177/19418744231223283 (hoshina2024vasculitisinthe pages 2-3)
• Sheikh TS, et al. Clinical/neuropath/radiologic characteristics of PCNSV (series). Frontiers in Neurology. Apr 2024. https://doi.org/10.3389/fneur.2024.1363985 (sheikh2024primarycnsvasculitis pages 1-2)

References

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12. (d’aniello2024thecontributionof pages 8-9): Serena D’Aniello, Arianna Rustici, Laura Ludovica Gramegna, Claudia Godi, Laura Piccolo, Mauro Gentile, Andrea Zini, Alessandro Carrozzi, Raffaele Lodi, Caterina Tonon, Massimo Dall’Olio, Luigi Simonetti, Raffaella Chieffo, Nicoletta Anzalone, and Luigi Cirillo. The contribution of vessel wall magnetic resonance imaging to the diagnosis of primary and secondary central nervous system vasculitis. Diagnostics, 14:927, Apr 2024. URL: https://doi.org/10.3390/diagnostics14090927, doi:10.3390/diagnostics14090927. This article has 12 citations.

13. (rice2026primarycentralnervous pages 2-4): Claire Rice and Neil Scolding. Primary central nervous system vasculitis: an update. Journal of Neurology, Mar 2026. URL: https://doi.org/10.1007/s00415-026-13727-y, doi:10.1007/s00415-026-13727-y. This article has 0 citations and is from a domain leading peer-reviewed journal.

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15. (NCT03166319 chunk 1): Vance Lehman. Vessel Wall Imaging for Diagnosis and Monitoring of Central Nervous System (CNS) Vasculitis. Mayo Clinic. 2017. ClinicalTrials.gov Identifier: NCT03166319

16. (NCT05168475 chunk 1): David Jayne. Biologics in Refractory Vasculitis: A Trial of Biologic Therapy for Refractory Primary Non-ANCA Associated Vasculitis. Cambridge University Hospitals NHS Foundation Trust. 2021. ClinicalTrials.gov Identifier: NCT05168475

17. (NCT00659776 chunk 1): Edward Neuwelt. MR, Histologic And EM Imaging Of Intravenous Ferumoxytol In Central Nervous System (CNS) Inflammation. Oregon Health and Science University. 2004. ClinicalTrials.gov Identifier: NCT00659776

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