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Vasculitis in children
Despina Eleftheriou1, Paul A Brogan
1Department of Paediatric Rheumatology, Institute of Child Health and Great Ormond St Hospital for Children, London, UK. d.eleftheriou@ich.ucl.ac.uk
Insights
Pediatric systemic vasculitides are rare but serious. This review covers childhood vasculitides, focusing on causes, genetics, and improving treatments like biologics, while noting long-term cardiovascular risks.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Vascular Medicine
Background:
- Primary systemic vasculitides in children are rare but impactful, causing significant morbidity and mortality.
- Understanding these conditions is crucial for timely diagnosis and management.
- Recent advancements in classification and treatment offer new hope for affected children.
Purpose of the Study:
- To provide a comprehensive overview of vasculitides predominantly affecting children.
- To detail specific childhood vasculitides like Kawasaki Disease and Henoch Schönlein purpura.
- To discuss emerging insights into genetic predispositions and infectious triggers.
Main Methods:
- Review of current literature on pediatric systemic vasculitides.
- Focus on diseases primarily affecting the pediatric population.
- Inclusion of recent proposals for new classification criteria.
Main Results:
- Infectious triggers are implicated in Kawasaki Disease and Henoch Schönlein purpura.
- Genetic polymorphisms may influence disease susceptibility and complications.
- Treatment strategies are evolving with immunosuppressants and biologic agents.
Conclusions:
- New classification criteria for childhood vasculitis are under validation.
- Long-term cardiovascular morbidity and endothelial health remain key concerns.
- International collaboration is vital for advancing research and improving outcomes in pediatric vasculitis.
Abstract:
Primary systemic vasculitides of the young are relatively rare diseases, but can have a significant morbidity and mortality. The purpose of this review is to provide an overview of the paediatric vasculitides. Vasculitides that predominantly affect children will be considered in more detail than vasculitic diseases that although are seen in children affect adults more commonly, such as the ANCA associated vasculitides. New classification criteria for childhood vasculitis have recently been proposed and are currently undergoing validation. Epidemiological clues continue to implicate infectious triggers in Kawasaki Disease and Henoch Schönlein purpura. Several genetic polymorphisms have now been described in the vasculitides that may be relevant in terms of disease predisposition or development of disease complications. Treatment regimens continue to improve, with the use of different immunosuppressive medications and newer therapeutic approaches such as biologic agents. However new challenges are looming in regards to the role of inflammation in endothelial health and the long term cardiovascular morbidity for children with primary systemic vasculitis. International multicenter collaboration is of utmost importance in order for us to further advance our understanding and improve the treatment and outcome of systemic vasculitis in the young.
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