Stroke frequency, associated factors, and clinical features in primary systemic vasculitis: a multicentric observational study

• Published in: Journal of neurology Vol. 271; no. 6; pp. 3309 - 3320
• Main Authors: Geraldes, Ruth, Santos, Monica, Ponte, Cristina, Craven, Anthea, Barra, Lillian, Robson, Joanna C., Hammam, Nevin, Springer, Jason, Henes, Jöerg, Hocevar, Alojzija, Putaala, Jukka, Santos, Ernestina, Rajasekhar, Liza, Daikeler, Thomas, Karadag, Omer, Costa, Andreia, Khalidi, Nader, Pagnoux, Christian, Canhão, Patrícia, Melo, Teresa Pinho e, Fonseca, Ana Catarina, Ferro, José M., Fonseca, João Eurico, Suppiah, Ravi, Watts, Richard A., Grayson, Peter, Merkel, Peter A., Luqmani, Raashid A.
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01-06-2024; Springer Nature B.V
• Subjects: Adult; Aged; Arteritis; Behcet's syndrome; Diagnosis; Female; Follow-Up Studies; Granulomatosis; Humans; Immunoglobulin A; Incidence; Ischemia; Male; Medicine; Medicine & Public Health; Middle Aged; Neurology; Neuroradiology; Neurosciences; Original Communication; Population studies; Prospective Studies; Risk Factors; Schonlein-Henoch purpura; Stroke; Stroke - diagnosis; Stroke - epidemiology; Stroke - etiology; Systemic vasculitis; Systemic Vasculitis - diagnosis; Systemic Vasculitis - epidemiology; Transient ischemic attack; Vascular diseases; Cerebrovascular event; Stroke; Primary systemic vasculitis; Transient ischaemic attack
• ISSN: 0340-5354; 1432-1459; 1432-1459
• DOI: 10.1007/s00415-024-12251-1

Objectives
The cerebral vessels may be affected in primary systemic vasculitis (PSV), but little is known about cerebrovascular events (CVEs) in this population. This study aimed to determine the frequency of CVEs at the time of diagnosis of PSV, to identify factors associated with CVEs in PSV, and to explore features and outcomes of stroke in patients with PSV.
Methods
Data from adults newly diagnosed with PSV within the Diagnostic and Classification Criteria in VASculitis (DCVAS) study were analysed. Demographics, risk factors for vascular disease, and clinical features were compared between patients with PSV with and without CVE. Stroke subtypes and cumulative incidence of recurrent CVE during a prospective 6-month follow-up were also assessed.
Results
The analysis included 4828 PSV patients, and a CVE was reported in 169 (3.50%, 95% CI 3.00–4.06): 102 (2.13% 95% CI 1.73–2.56) with stroke and 81 (1.68% 95% CI 1.33–2.08) with transient ischemic attack (TIA). The frequency of CVE was highest in Behçet’s disease (9.5%, 95% CI 5.79–14.37), polyarteritis nodosa (6.2%, 95% CI 3.25–10.61), and Takayasu’s arteritis (6.0%, 95% CI 4.30–8.19), and lowest in microscopic polyangiitis (2.2%, 95% CI 1.09–3.86), granulomatosis with polyangiitis (2.0%, 95% CI 1.20–3.01), cryoglobulinaemic vasculitis (1.9%, 95% CI 0.05–9.89), and IgA-vasculitis (Henoch-Schönlein) (0.4%, 95% CI 0.01–2.05). PSV patients had a 11.9% cumulative incidence of recurrent CVE during a 6-month follow-up period.
Conclusion
CVEs affect a significant proportion of patients at time of PSV diagnosis, and the frequency varies widely among different vasculitis, being higher in Behçet’s. Overall, CVE in PSV is not explained by traditional vascular risk factors and has a high risk of CVE recurrence.