| Treatment/strategy | Phase (induction/maintenance) | Evidence type | Key quantitative outcome | Notes | Source |
|---|---|---|---|---|---|
| Intravenous methylprednisolone | Induction | Single-center retrospective CNS vasculitis cohort (n=44) | Used in 28/44 patients (63.6%); overall cohort mortality 9/44 (20.5%), primary 16.0%, secondary 26.3% | Predominant induction therapy across CNS vasculitis etiologies; outcome figures are cohort-level, not steroid-specific (pqac-00000002, pqac-00000004) | Hoshina 2024, Neurohospitalist, published Dec 2024, https://doi.org/10.1177/19418744231223283 (pqac-00000002, pqac-00000004) |
| High-dose corticosteroids ± cyclophosphamide | Induction | 2023 European guideline / expert consensus | No pooled effect size established; evidence judged very low quality | ESO guideline states induction recommendations reflect major uncertainty due to sparse evidence; management should be multidisciplinary in expert centers (pqac-00000000) | Pascarella et al. 2023, European Stroke Journal, Oct 2023, https://doi.org/10.1177/23969873231190431 (pqac-00000000) |
| Corticosteroids plus cyclophosphamide | Induction | Single-center retrospective PCNSV series (n=5) / narrative clinical update | No formal response rate reported; 2 improved, 2 stable, 1 deteriorated over median 56 months follow-up | Authors describe prompt immunotherapy, typically corticosteroids plus cyclophosphamide, as recommended induction; report states immunotherapy significantly improved clinical and radiologic outcomes, but not with drug-specific percentages (pqac-00000003) | Sheikh et al. 2024, Frontiers in Neurology, Apr 2024, https://doi.org/10.3389/fneur.2024.1363985 (pqac-00000003) |
| Cyclophosphamide as adjunct to steroids | Induction / adjunct | Single-center retrospective CNS vasculitis cohort (n=44) | Most used adjunctive therapy; subgroup use reported as 47.1%, 25%, and 66.7% in cohort summaries | Frequently paired with IV methylprednisolone in real-world practice; exact efficacy by subgroup not isolated in retrieved evidence (pqac-00000002) | Hoshina 2024, https://doi.org/10.1177/19418744231223283 (pqac-00000002) |
| Intravenous cyclophosphamide | Induction | Comparative retrospective cohort, PACNS vs secondary CNS vasculitis (20 vs 20) | Nearly half of PACNS patients received IV cyclophosphamide for induction; mortality/relapse/outcomes did not differ between PACNS and SACNS | PACNS showed more seizures and pseudotumoral lesions, but outcome separation by induction regimen was not demonstrated (pqac-00000006, pqac-00000007) | Scoppettuolo et al. 2025, Frontiers in Neurology, Oct 2025, https://doi.org/10.3389/fneur.2025.1602427 (pqac-00000006, pqac-00000007) |
| Cyclophosphamide within 3 months (“early intensive treatment”) | Early intensive induction | Retrospective biopsy-proven small-vessel PCNSV cohort (n=26; non-ABRA subgroup n=19) | Remission in 12/12 (100%) early-intensive group vs 11/14 (78.6%) escalation group; median time to remission 5 vs 19 months; HR 0.24, 95% CI 0.10–0.63, p<0.005; overall relapse 19% | Strongest quantitative treatment-outcome signal in retrieved evidence; supports earlier aggressive therapy in sv-PCNSV, though from 2025 source | Reddy et al. 2025, Neurology Neuroimmunology & Neuroinflammation, Jul 2025, https://doi.org/10.1212/NXI.0000000000200397 (pqac-00000014) |
| Glucocorticoids alone or with azathioprine/mycophenolate/methotrexate (“escalation treatment”) | Initial non-intensive induction / early maintenance | Retrospective biopsy-proven small-vessel PCNSV cohort | Remission achieved in 11/14 (78.6%); slower median time to remission (19 months) than early cyclophosphamide group | Comparator arm in Reddy 2025; suggests less rapid disease control than early intensive cyclophosphamide (pqac-00000014) | Reddy et al. 2025, https://doi.org/10.1212/NXI.0000000000200397 (pqac-00000014) |
| Azathioprine | Maintenance | Cohort + review/expert opinion | Used as maintenance in real-world cohort; in PACNS/SACNS comparison, azathioprine was the commonest maintenance agent; no regimen-specific remission rate reported | Common steroid-sparing maintenance option after induction; often continued for at least 2 years in expert practice summaries (pqac-00000004, pqac-00000007) | Hoshina 2024, https://doi.org/10.1177/19418744231223283; Scoppettuolo 2025, https://doi.org/10.3389/fneur.2025.1602427 (pqac-00000004, pqac-00000007) |
| Mycophenolate mofetil | Maintenance | Cohort + review/expert opinion | Used in maintenance; no drug-specific response percentage reported in retrieved evidence | Listed among maintenance/steroid-sparing agents after induction in PCNSV/PACNS (pqac-00000003, pqac-00000004, pqac-00000005, pqac-00000007) | Sheikh 2024, https://doi.org/10.3389/fneur.2024.1363985; Hoshina 2024, https://doi.org/10.1177/19418744231223283; Rice 2026, https://doi.org/10.1007/s00415-026-13727-y (pqac-00000003, pqac-00000004, pqac-00000005, pqac-00000007) |
| Methotrexate | Maintenance | Review/expert opinion and clinical series | No quantitative outcome reported in retrieved evidence | Included among less toxic post-induction maintenance immunosuppressants (pqac-00000003, pqac-00000005, pqac-00000007) | Sheikh 2024, https://doi.org/10.3389/fneur.2024.1363985; Rice 2026, https://doi.org/10.1007/s00415-026-13727-y; Scoppettuolo 2025, https://doi.org/10.3389/fneur.2025.1602427 (pqac-00000003, pqac-00000005, pqac-00000007) |
| Rituximab | Maintenance / alternative immunomodulator | Real-world cohort + review/expert opinion | Used in maintenance cohorts; no PACNS-specific response rate quantified in retrieved evidence | Considered a steroid-sparing or refractory-disease option; case-report/series evidence exists, but robust comparative data lacking (pqac-00000004, pqac-00000005, pqac-00000007) | Hoshina 2024, https://doi.org/10.1177/19418744231223283; Rice 2026, https://doi.org/10.1007/s00415-026-13727-y; Scoppettuolo 2025, https://doi.org/10.3389/fneur.2025.1602427 (pqac-00000004, pqac-00000005, pqac-00000007) |
| Prednisone with maintenance immunosuppressants | Maintenance / taper | Retrospective cohort | Often used concurrently with cyclophosphamide, rituximab, azathioprine, or mycophenolate; no isolated efficacy estimate | Reflects common real-world tapering strategy after IV steroid induction (pqac-00000004) | Hoshina 2024, https://doi.org/10.1177/19418744231223283 (pqac-00000004) |
| Plasma exchange (PLEX) | Adjunct / rescue | Small cohort observation | 2 PACNS cases received PLEX with favorable outcomes | Evidence is anecdotal and insufficient for routine recommendation (pqac-00000002) | Hoshina 2024, https://doi.org/10.1177/19418744231223283 (pqac-00000002) |
| Maintenance immunosuppression after remission | Maintenance strategy | Review/expert opinion | No numeric estimate in retrieved excerpt; cited as improving long-term outcomes in prior literature referenced by review | Rice 2026 notes maintenance with azathioprine, methotrexate, or mycophenolate after induction, extrapolated largely from systemic vasculitis evidence (pqac-00000005) | Rice & Scolding 2026, Journal of Neurology, Mar 2026, https://doi.org/10.1007/s00415-026-13727-y (pqac-00000005) |


*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.*