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Cerebrovascular involvement in systemic childhood vasculitides

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Abstract

Pediatric vasculitides sometimes involve central nervous system (CNS). The manifestations are diverse, ranging from headache, seizures, vertigo, ataxia, behavioral changes, neuropsychiatric symptoms, consciousness disorders, and even cerebrovascular (CV) accidents that may lead to irreversible impairment and even death. Stroke, on the other hand despite the great progress in prevention and treatment, is still one of the leading causes of morbidity and mortality in the general population. The aim of this article was to summarize CNS manifestations and CV issues observed in primary pediatric vasculitides and the current knowledge of etiology and CV risk factors, preventive strategies, and therapeutic options in this target patient population. Pathophysiological links reveal similar immunological mechanisms involved in both pediatric vasculitides and CV events with endothelial injury and damage being the central point. From the clinical point of view, CV events in pediatric vasculitides were associated with increased morbidity and poor prognosis. If damage has already occurred, the therapeutic approach consists of good management of the vasculitis itself, antiplatelet and anticoagulation therapy, and early rehabilitation. Risk factors for acquiring cerebrovascular disease (CVD) and stroke, particularly hypertension and early atherosclerotic changes, already begin in childhood, with vessel wall inflammation contributing itself, once more emphasizing that appropriate preventive measures are certainly necessary in pediatric vasculitis population to improve their long-term outcome.

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Authors and Affiliations

  1. Department of Pediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia

    Martina Held, Mario Sestan, Nastasia Kifer & Marija Jelusic

  2. Division of Clinical Immunology, Rheumatology and Allergology, Centre of Reference for Paediatric and Adolescent Rheumatology of Ministry of Health of the Republic Croatia, University Hospital Centre Zagreb, Kispaticeva 12, 10 000, Zagreb, Croatia

    Marija Jelusic

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  2. Mario Sestan
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MH reviewed the literature, analyzed the data, and wrote much of the manuscript. MS and NK reviewed the literature and wrote parts of the manuscript. MJ contributed to the conception and design of the work; she reviewed and revised the manuscript and supervised the work. All authors read and approved the final manuscript to be published. All co-authors are full responsible for all aspects of the study and the final manuscript. All authors accept responsibility for the accuracy and integrity of the final manuscript as submitted.

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Key Messages

• Pediatric vasculitides are rare, but possible cause of stroke in children and adolescents

• Endothelial injury plays central role in both pediatric vasculitides and cerebrovascular diseases (CVDs), including stroke

• The healthcare approach to a pediatric vasculitis patient who developed CVD includes three key steps: vasculitis specific treatment, CVD management and implementation of preventive measures

Part of the Topical Collection entitled ‘Cardiovascular Issues in Rheumatic Diseases’

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Held, M., Sestan, M., Kifer, N. et al. Cerebrovascular involvement in systemic childhood vasculitides. Clin Rheumatol 42, 2733–2746 (2023). https://doi.org/10.1007/s10067-023-06552-5

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