| Concept | Identifier system | Identifier/code | Notes/source |
|---|---|---|---|
| Primary angiitis of the central nervous system | Preferred disease name in guideline/literature | PACNS | Used as the main term in the 2023 European Stroke Organisation guideline title and throughout the retrieved CNS vasculitis literature; primary form is defined as isolated CNS vasculitis without systemic disease (https://doi.org/10.1177/23969873231190431; Oct 2023) (pqac-00000000, pqac-00000004) |
| Primary CNS vasculitis | Synonym / literature term | PCNSV | Used synonymously with PACNS in 2024-2026 retrieved studies/reviews; D’Aniello 2024 and Sheikh 2024 use “primary CNS vasculitis/PCNSV” for the primary form (https://doi.org/10.3390/diagnostics14090927; Apr 2024; https://doi.org/10.3389/fneur.2024.1363985; Apr 2024) (pqac-00000003, pqac-00000010) |
| CNS vasculitis | Broad disease term | CNSV | Broad umbrella term covering primary and secondary CNS vasculitis; Hoshina 2024 explicitly stratified cases into primary and secondary CNS vasculitis (https://doi.org/10.1177/19418744231223283; Dec 2024) (pqac-00000002, pqac-00000004) |
| Primary CNS vasculitis / primary angiitis of the CNS | Diagnostic category (Birnbaum & Hellmann framework cited in cohort) | Definite / Probable | Hoshina 2024 notes PACNS is “definite” with tissue confirmation and “probable” without biopsy if imaging/CSF are supportive; ESO 2023 emphasizes histopathology as gold standard (https://doi.org/10.1177/19418744231223283; Dec 2024; https://doi.org/10.1177/23969873231190431; Oct 2023) (pqac-00000000, pqac-00000004) |
| Large-vessel primary angiitis of the CNS | Guideline subclassification | LV-PACNS | ESO 2023 explicitly distinguishes LV-PACNS from SV-PACNS (https://doi.org/10.1177/23969873231190431; Oct 2023) (pqac-00000000) |
| Small-vessel primary angiitis of the CNS | Guideline subclassification | SV-PACNS | ESO 2023 explicitly distinguishes SV-PACNS from LV-PACNS (https://doi.org/10.1177/23969873231190431; Oct 2023) (pqac-00000000) |
| Secondary CNS vasculitis | Disease category / comparator | SCNSV / SACNS | Retrieved sources use both SCNSV and SACNS for vasculitis secondary to infection, connective tissue disease, malignancy, or systemic vasculitis (https://doi.org/10.3390/diagnostics14090927; Apr 2024; https://doi.org/10.3389/fneur.2025.1602427; Oct 2025) (pqac-00000007, pqac-00000010) |
| Inflammatory cerebral amyloid angiopathy | Primary CNS vasculitis-related entity in cohort | inflammatory CAA | Hoshina 2024 describes inflammatory CAA, including ABRA, as a primary CNS vasculitis entity with distinct biopsy features (https://doi.org/10.1177/19418744231223283; Dec 2024) (pqac-00000004) |
| Amyloid-beta related angiitis | Synonym / subtype label | ABRA | Included within inflammatory CAA/small-vessel predominant primary CNS vasculitis in retrieved cohort evidence (https://doi.org/10.1212/nxi.0000000000200397; Jul 2025; https://doi.org/10.1177/19418744231223283; Dec 2024) (pqac-00000004) |
| CNS vasculitis | ICD-9 | 437.4 | Explicitly reported in Hoshina 2024 as an ICD code used for case identification (https://doi.org/10.1177/19418744231223283; Dec 2024) (pqac-00000002) |
| Cerebral arteritis, not elsewhere classified | ICD-10 | I67.7 | Explicitly reported in Hoshina 2024 as an ICD-10 code used for CNS vasculitis case identification (https://doi.org/10.1177/19418744231223283; Dec 2024) (pqac-00000002) |
| CNS vasculitis / PACNS | MeSH | not found in retrieved sources | No MeSH identifier was explicitly present in the provided evidence (pqac-00000000, pqac-00000004) |
| CNS vasculitis / PACNS | MONDO | not found in retrieved sources | No MONDO identifier was explicitly present in the provided evidence (pqac-00000000, pqac-00000004) |
| CNS vasculitis / PACNS | Orphanet | not found in retrieved sources | No Orphanet identifier was explicitly present in the provided evidence (pqac-00000000, pqac-00000004) |
| CNS vasculitis / PACNS | OMIM | not found in retrieved sources | No OMIM identifier was explicitly present in the provided evidence (pqac-00000000, pqac-00000004) |
| CNS vasculitis / PACNS | ICD-11 | not found in retrieved sources | No ICD-11 code was explicitly present in the provided evidence (pqac-00000000, pqac-00000004) |


*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.*