Hamburger Zentrum für Kinder- und Jugendrheumatologie, Dehnhaide 120, Hamburg 22081, Germany.
Rheum Dis Clin North Am. 2013 Nov;39(4):905-20. doi: 10.1016/j.rdc.2013.05.003. Epub 2013 Jul 16.
Juvenile localized scleroderma (jLS) and juvenile systemic sclerosis (jSS) are both orphan diseases, with jLS around 10 times more frequent than jSS. In recent years the time gap between the appearance of symptoms and diagnosis has become significantly shorter. This review focuses on the new classifications of jSS and jLS, and on the developments and adaptations of the outcome measures for certain organ involvements whereby progress has been made regarding pediatric patients.
局限性幼年型硬皮病(jLS)和系统性幼年型硬皮病(jSS)均为孤儿病,jLS 比 jSS 发病率高约 10 倍。近年来,症状出现与诊断之间的时间间隔明显缩短。本文主要关注 jSS 和 jLS 的新分类,以及特定器官受累的评估方法的发展和适应性变化,在儿科患者方面取得了一定进展。