Presentation and Classification of Rare Primary Vasculitides in Children

Primary vasculitides, where blood vessels are the primary site of inflammation, with the exception of Henoch Schönlein purpura (HSP) and Kawasaki’s disease (KD) are rare in childhood but when present, cause significant morbidity and mortality. This review provides an update on classification and presentation of rare vasculitides in children, through considering our experience in three cases in context with the current literature. We have demonstrated the challenges to diagnosis and how diagnosis can easily be missed if vasculitides are not carefully considered and looked for. The introduction of classification criteria has been important to allow more research into rare paediatric vasculitides but development of diagnostic criteria may be useful. Vasculitides should be considered in all patients presenting with multi-system features.