VASCULITIDES IN CHILDHOOD

Primary systemic vasculitides in children are relatively rare diseases. In most cases, they have an unknown etiology and are defined as the presence of inflammation in the blood vessel wall. Establishing the diagnosis of vasculitis is oft en challenging, since the disorder is multisystem in nature with mostly nonspecific symptoms. The last 10 years have seen significant advances in the field of pediatric vasculitis: the development and validation of classification criteria as well as tools to assess clinical disease activity and disease outcome, the inclusion of pediatric patients in international multicentre randomized controlled trial designs for therapies of vasculitis, clinical trials for very rare pediatric vasculitides, and identification of a special group of monogenic vasculitides. In this paper we want to introduce readers to the giant leap in the field of pediatric vasculitis as a result of the hard work of pediatric rheumatologists in vasculitis work groups.