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

Journal: Journal of neurology

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Affiliated Institutions:  Neurology Department, Wexham Park Hospital, Frimley Health Foundation Trust, Slough, UK. ruth.geraldes@ndcn.ox.ac.uk. Serviço de Neurologia, Departamento de Neurociências e Saúde Mental, Centro Hospitalar Universitário Lisboa Norte, Lisbon, Portugal. Rheumatology and Metabolic Bone Diseases Department, Centro Hospitalar Universitário Lisboa Norte, Lisbon Academic Medical Centre, Lisbon, Portugal. Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, Nuffield Orthopaedic Centre, Botnar Research Centre, University of Oxford, Oxford, UK. Lawson Health Research Institute, London, ON, Canada. Centre for Health and Clinical Research, University of the West of England, Bristol, UK. Rheumatology Department, Faculty of Medicine, Assiut University, Assiut, Egypt. University of Kansas Medical Centre Institute, Lawrence, Kansas, KS, USA. Centre for Interdisciplinary Clinical Immunology, Rheumatology and Auto-Inflammatory Diseases, Department of Internal Medicine II (Oncology, Haematology, Immunology and Rheumatology), University Hospital Tuebingen, Tuebingen, Germany. University Medical Centre Ljubljana, Ljubljana, Slovenia. Helsinki University Central Hospital, Helsinki, Finland. Hospital de Santo António, Centro Hospitalar Universitário do Porto, Porto, Portugal. NIMS, Hyderabad, India. Department of Rheumatology and Clinical Research, University Hospital, Basel, Switzerland. Division of Rheumatology, Department of Internal Medicine, Vasculitis Research Center, Hacettepe University School of Medicine, Ankara, Turkey. Centro Hospitalar Universitário de São João, Porto, Portugal. Joseph's Healthcare Hamilton and McMaster University, Hamilton, ON, Canada. Mount Sinai Hospital, Toronto, Canada. Instituto de Medicina Molecular, Faculdade de Medicina da Universidade de Lisboa, Lisbon, Portugal. Te Whatu Ora-Health, Wellington, New Zealand. Norwich Medical School, Norwich, UK. National Institutes of Health, NIAMS Vasculitis Translational Research Program, Bethesda, USA. Division of Rheumatology, Department of Medicine, University of Pennsylvania, Philadelphia, USA.

Abstract summary 

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.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.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.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.

Authors & Co-authors:  Geraldes Santos Ponte Craven Barra Robson Hammam Springer Henes Hocevar Putaala Santos Rajasekhar Daikeler Karadag Costa Khalidi Pagnoux Canhão Melo Fonseca Ferro Fonseca Suppiah Watts Grayson Merkel Luqmani

Study Outcome 

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Statistics
Citations :  Boot E, Ekker MS, Putaala J et al (2020) Ischaemic stroke in young adults: a global perspective. J Neurol Neurosurg Psychiatry 91:411–417. https://doi.org/10.1136/jnnp-2019-322424
Authors :  29
Identifiers
Doi : 10.1007/s00415-024-12251-1
SSN : 1432-1459
Study Population
Male,Female
Mesh Terms
Other Terms
Cerebrovascular event;Primary systemic vasculitis;Stroke;Transient ischaemic attack
Study Design
Study Approach
Country of Study
Publication Country
Germany