Division of Pediatric Rheumatology, Department of Woman and Child Health, University of Padua, Padua, Italy.
Best Pract Res Clin Rheumatol. 2017 Aug;31(4):576-595. doi: 10.1016/j.berh.2018.02.004. Epub 2018 Mar 27.
Juvenile scleroderma with its two varieties, juvenile localized scleroderma and systemic sclerosis (SSc), represents the third most frequent rheumatic disease in childhood. In juvenile SSc, new developments have been recently reported in the fields of classification and monitoring. The introduction of new classification criteria for adult SSc has stimulated new ideas on how to improve the performance of the provisional 2007 PRES/ACR/EULAR pediatric criteria. The introduction of a multidimensional severity score, named "J4S," which includes parameters on growth, skin, and internal organ involvement, has improved the approach to the patients in the daily practice to guide decision-making. In localized scleroderma, the wider application of clinical and instrumental scoring systems has greatly improved both assessment and monitoring. Finally, a multicenter consensus statement and long-term follow-up studies have confirmed the important role of methotrexate for the treatment.
幼年型硬皮病及其两种类型，即局限性幼年型硬皮病和系统性硬皮病（SSc），是儿童时期第三大常见的风湿性疾病。在幼年型硬皮病中，分类和监测领域最近有新的发展。成人 SSc 新分类标准的引入激发了如何提高临时 2007PRES/ACR/EULAR 儿科标准性能的新想法。引入一种多维严重程度评分，称为“J4S”，它包括生长、皮肤和内脏器官受累的参数，这提高了在日常实践中对患者的处理方法，以指导决策。在局限性硬皮病中，临床和仪器评分系统的更广泛应用极大地提高了评估和监测。最后，多中心共识声明和长期随访研究证实了甲氨蝶呤治疗的重要作用。
