| Abstract: | Systemic vasculitis is characterized by blood vessel inflammation which may lead to tissue injury from vascular stenosis, occlusion, aneurysm, and/or rupture. Apart from relatively common vasculitides such as IgA Vasculitis (IgAV; previously referred to as Henoch-Sch?nlein Purpura [HSP]) and Kawasaki disease (KD), most of the primary vasculitic syndromes are rare in childhood, but are associated with significant morbidity and mortality. The cause of the majority of vasculitides is unknown, although it is likely that a complex interaction between environmental factors such as infections and inherited host responses trigger the disease and determine the vasculitis phenotype. Several genetic polymorphisms in vasculitis have now been described. Treatment regimens continue to improve, with the use of different immunosuppressive medications and newer therapeutic approaches such as biologic agents. Randomized control studies involving predominantly adults have recently recruited children with vasculitis too; but rare disease trial design is required for paediatric specific trials. The SHARE (Single-Hub Access for Pediatric Rheumatology in Europe) project has recently provided guidance on management of rare paediatric rheumatic diseases including the vasculitides. This article provides an overview of paediatric vasculitides with emphasis on presenting features, current insights on aetiopathogenesis and treatment advances. |
