Division of Pediatric Rheumatology, Department of Pediatrics, University of Padua, Padua, Italy.
Curr Opin Rheumatol. 2013 Sep;25(5):643-50. doi: 10.1097/BOR.0b013e3283641f61.
Scleroderma, in its localized and systemic presentation, represents the third most frequent rheumatic condition in childhood after juvenile idiopathic arthritis and systemic lupus erythematosus. Early diagnosis, appropriate assessment and effective treatment are crucial to improve the long-term outcome.
Recent studies, concerning histopathology and clinical associations with other conditions, open new horizons on the etiopathogenesis of scleroderma. New developments have been also reached in the field of outcome measures. In juvenile localized scleroderma (JLS), new techniques such as Doppler and laser Doppler imaging have shown their usefulness for the daily monitoring of the patients. In juvenile systemic sclerosis (JSSc), a new severity score has been developed and needs to be validated in future trials. Finally, a randomized, double-blind controlled trial, a multicenter consensus statement and long-term follow-up studies have confirmed the important role of methotrexate (MTX) for the treatment of JLS.
Studies over recent years highlighted the role of imaging as outcome measures for JLS and introduced a severity score for JSSc. New studies on MTX confirmed its important role for the treatment of JLS.
硬皮病在局限性和系统性表现中，是儿童时期仅次于幼年特发性关节炎和系统性红斑狼疮的第三大常见风湿性疾病。早期诊断、适当评估和有效治疗对于改善长期预后至关重要。
最近的研究涉及组织病理学和与其他疾病的临床关联，为硬皮病的病因发病机制开辟了新的视野。在结局评估领域也取得了新的进展。在局限性幼年型硬皮病（JLS）中，新的技术如多普勒和激光多普勒成像已经显示出其在患者日常监测中的有用性。在幼年系统性硬皮病（JSSc）中，已经开发出一种新的严重程度评分，需要在未来的试验中进行验证。最后，一项随机、双盲对照试验、多中心共识声明和长期随访研究证实了甲氨蝶呤（MTX）在 JLS 治疗中的重要作用。
近年来的研究强调了影像学作为 JLS 结局评估的作用，并引入了 JSSc 的严重程度评分。关于 MTX 的新研究证实了其在 JLS 治疗中的重要作用。
