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EULAR/PReS Online Course in Paediatric Rheumatology (chapter: Vasculitis)

Most of the primary vasculitic syndromes, apart from IgA vasculitis and Kawasaki disease, are rare in childhood, but are associated with significant morbidity and mortality. Classification criteria for childhood vasculitis have been developed, but should not be misused as diagnostic criteria. The cause of most 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. Monogenic forms of vasculitis are increasingly described. Treatment regimens continue to improve, with the use of different immunosuppressive medications and newer therapeutic approaches such as biological agents and development of more targeted therapies, although the evidence base remains limited in pediatric practice.

Vasculitis

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