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

Vasculitis is an autoimmune disorder causing blood vessel inflammation, potentially affecting the central nervous system. This review explores neuropsychiatric manifestations with a focus on cognitive impairment in patients with vasculitis. Embase, PubMed, Scopus, and PsycINFO were searched up to December 31st, 2023, for studies of primary vasculitis reporting on cognitive impairment. Case reports, reviews, and case series with <5 patients were excluded. Study quality was evaluated using the Newcastle Ottawa Scale. From the 4,249 identified studies, 43 met the inclusion criteria, comprising 1,216 cases of PCNSV, 481 of ANCA-associated vasculitis (AAV), 151 of giant cell arteritis (GCA), 28 of Takayasu arteritis (TAK), 22 of polyarteritis nodosa (PAN), 32 of Behçet's disease (BD), and 54 of other vasculitis types. Patients' ages ranged from 7.1-75.6 years and 11-100 % were female. Most studies were single-center retrospective studies of good or fair quality. Cognitive impairment was noted in 275/750 (11-62 %) of adult-onset PCNSV, 126/433 (10-54 %) of childhood-onset PCNSV, 62/157 (12-61 %) of AAV, 4/12 (20-43 %) of GCA, and 261/486 (20-67 %) of other vasculitis cases. Standardized neuropsychological testing was done in 12/43 (28 %) of studies. A minority of studies investigated factors associated with neuropsychiatric manifestations or response to treatment. These studies reported associations with MRI abnormalities and with worse prognosis and/or higher disease activity. Most patients were reported to improve after immunosuppressive therapy but have residual neuropsychiatric symptoms. Cognitive impairment and other neuropsychiatric manifestations are commonly reported in vasculitis, highlighting the need for routine neuropsychological assessments.