Jelušić Marija, Frković Marijan
Reumatizam. 2016;63 Suppl 1:80-5.
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.
儿童原发性系统性血管炎是相对罕见的疾病。在大多数情况下，其病因不明，定义为血管壁存在炎症。由于该疾病本质上是多系统的且大多症状不具特异性，因此确立血管炎的诊断往往具有挑战性。过去十年，儿科血管炎领域取得了重大进展：分类标准以及评估临床疾病活动度和疾病转归的工具得以开发和验证；儿科患者被纳入血管炎治疗的国际多中心随机对照试验设计；针对非常罕见的儿科血管炎开展了临床试验；还识别出了一组特殊的单基因血管炎。在本文中，我们想向读者介绍儿科血管炎领域因儿科风湿病学家在血管炎工作组的辛勤工作而实现的巨大飞跃。
