Primarni sistemski vaskulitisi u djece relativno su rijetke bolesti, većinom nepoznate etiologije, kojima
je zajedničko obilježje upala u stijenci krvne žile. Teško ih je dijagnosticirati jer su zahvaćeni mnogi organi, a simptomi
su uglavnom nespecifi čni. U posljednjih 10 godina postignut je znatan napredak u području vaskulitisa u dječjoj dobi:
defi nirani su i validirani klasifi kacijski kriteriji, razvijeni i validirani upitnici za procjenu aktivnosti bolesti i ishoda,
pedijatrijski bolesnici uključeni su u međunarodna multicentrična istraživanja vezana uz terapiju vaskulitisa, započeta
su klinička istraživanja izuzetno rijetkih vaskulitisa i izdvojena je zasebna skupina rijetkih monogenskih vaskulitisa.
Ovim radom želimo upoznati čitaoce s velikim iskorakom u području vaskulitisa u dječjoj dobi, nastalim na osnovi
mukotrpnog rada pedijatrijskih reumatologa u radnim skupinama za vaskulitise.Primary systemic vasculitides in children are relatively rare diseases. In most cases, they have an unknown
etiology and are defi ned as the presence of infl ammation in the blood vessel wall. Establishing the diagnosis of
vasculitis is oft en challenging, since the disorder is multisystem in nature with mostly nonspecifi c symptoms. Th e last
10 years have seen signifi cant advances in the fi eld of pediatric vasculitis: the development and validation of classifi cation
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 identifi cation of a special group of monogenic vasculitides. In this paper we want to introduce
readers to the giant leap in the fi eld of pediatric vasculitis as a result of the hard work of pediatric rheumatologists
in vasculitis work groups
