Vasculitis & polymyalgia rheumatica

Baseline features and functional outcomes in primary central nervous system vasculitis: development and validation of a prognostic model

Rheumatology (Oxford)Published Prognostic model development and validation study

Authors' conclusion

Baseline clinical and imaging variables may predict 12-month functional independence in adults with PCNSV.

Study context

Population
Adults with primary central nervous system vasculitis (PCNSV), COVAC' cohort (mean age 48 years; 41% female)67 (33%) biopsy-diagnosed
Participants
206 adults
Outcome
Functional independence (modified Rankin Scale 0-2) after start of corticosteroids or immunosuppressants
Follow-up
12 months
Methodological detailsmulticentre, international cohort, external validation in single-centre cohort from India, multivariable analyses, AUC-ROC
  • Multicentre, international cohort
  • External validation in single-centre cohort from India
  • Multivariable analyses
  • AUC-ROC

Performance

As reported in the abstract

Functional independence (mRS 0-2) and baseline prognostic factors
Functionally independent at 12 months
135 (66%)
≥1 intracranial stenosis on CT- or MR-angiogram (favourable), OR
2.99 (1.23-7.68) (95% CI)
Headache (favourable), OR
2.67 (1.31-5.59) (95% CI)
Altered level of consciousness (unfavourable), OR
0.09 (0.02-0.31) (95% CI)
≥1 acute brain infarct (unfavourable), OR
0.12 (0.04-0.33) (95% CI)
Cognitive impairment (unfavourable), OR
0.22 (0.10-0.46) (95% CI)
Five-variable model AUC, derivation cohort
0.80 (0.74-0.87) (95% CI)
Five-variable model AUC, validation cohort
0.67 (0.54-0.81) (95% CI)

Further findings

Death · 12 months

Died
12 (6%)

Original publication

Baseline features and functional outcomes in primary central nervous system vasculitis: development and validation of a prognostic model. Rheumatology (Oxford). Published 10 November 2025. doi:10.1093/rheumatology/keaf590

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