Vasculitis & polymyalgia rheumatica

Cognitive impairment and other neuropsychiatric manifestations in patients with primary vasculitis: a systematic review

Semin Arthritis RheumPublished Systematic review

Authors' conclusion

Cognitive impairment and other neuropsychiatric manifestations are commonly reported in vasculitis

Study context

Population
Patients with primary vasculitis (PCNSV, ANCA-associated vasculitis (AAV), giant cell arteritis (GCA), TAK, polyarteritis nodosa (PAN), Behçet's disease (BD), other types); ages 7.1–75.6 years, 11–100 % female43 studies
Participants
750 / 433 / 157 / 12 / 486
Comparison
Adult-onset PCNSV vs Childhood-onset PCNSV vs AAV vs GCA vs Other vasculitis
  • Adult-onset PCNSVn = 750
  • Childhood-onset PCNSVn = 433
  • AAVn = 157
  • GCAn = 12
  • Other vasculitisn = 486
Outcome
Cognitive impairment
Methodological detailsNewcastle Ottawa Scale quality assessment, Most included studies single-center retrospective, good or fair quality
  • Newcastle Ottawa Scale quality assessment
  • Most included studies single-center retrospective, good or fair quality

Pooled result

As reported in the abstract

Adult-onset PCNSV

11-62 %

275 of 750

Childhood-onset PCNSV

10-54 %

126 of 433

AAV

12-61 %

62 of 157

GCA

20-43 %

4 of 12

Other vasculitis

20-67 %

261 of 486

Further pooled results

Standardized neuropsychological testing done

Studies using standardized neuropsychological testing
12/43 (28 %)

Factors associated with neuropsychiatric manifestations (minority of studies)

Associations with MRI abnormalities and with worse prognosis and/or higher disease activity

Response to immunosuppressive therapy

Most patients improved but had residual neuropsychiatric symptoms

Original publication

Cognitive impairment and other neuropsychiatric manifestations in patients with primary vasculitis: a systematic review. Semin Arthritis Rheum. Published 5 August 2025. doi:10.1016/j.semarthrit.2025.152806

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