11.5 - Vasculitis

Abstract

Childhood vasculitides are a heterogeneous group of disorders that manifest in multisystem disease characterised by inflammation of blood vessels and may lead to tissue ischaemia and necrosis. Most forms of vasculitis are rare in childhood; primary systemic vasculitis (PSV) affects less than 25 per 100,000 children, with Henoch Schonlein purpura (HSP), now known as IgA vasculitis and Kawasaki disease (KD) being the two commonest. Other forms of vasculitis, although rare, are important as they are often associated with significant morbidity and mortality. With the wider use of immunosuppressive agents, both mortality and morbidity rates have improved, but many patients still develop significant treatment and disease-related morbidities, and mortality remains high. In 2005, this led to the European League Against Rheumatism/Pediatric Rheumatology European Society vasculitis working group to propose new vasculitis classification criteria for children. In 2008, at the Ankara Consensus Conference formal validation of these criteria was reached, and in 2010 the data were published with highly sensitive and specific validated criteria for HSP, polyarthritis nodosa (PAN), granulomatosis with polyangiitis (GPA) and Takayasu arteritis (TA) being established. With regard to microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA) and other forms of childhood vasculitides, there are few cases available to be able to validate criteria for these forms of vasculitis in children.