Department of Woman's and Child's Health, University of Padua, Via Giustiniani 3, 35128, Padua, Italy.
Curr Rheumatol Rep. 2020 Jun 26;22(8):45. doi: 10.1007/s11926-020-00910-x.
Treatment of scleroderma in children is challenging since little is known about its pathogenesis. Herein, we review the most recent evidence regarding the treatment of juvenile scleroderma.
According to the recent recommendations for Pediatric Rheumatology in Europe (SHARE), systemic treatment in localized scleroderma is needed when there is a risk for disability, such as in generalized or pansclerotic morphea and progressive linear scleroderma. In juvenile systemic sclerosis, the introduction of the severity score, J4S, has standardized the assessment of the patients in the daily practice and allowed a more tailored therapeutic approach. Since, to date, no clinical trial is available in JSSc, due to its rarity, the treatment is based on adults' experience. The recent recommendations for juvenile scleroderma represent an important instrument to standardize the treatment approach, confirm the role of methotrexate, and open new windows for effective experimental treatments, such as mycophenolate mofetil and biological agents, for severe or refractory cases.
儿童硬皮病的治疗具有挑战性，因为其发病机制知之甚少。本文综述了关于儿童硬皮病治疗的最新证据。
根据欧洲儿科风湿病学的最新建议（SHARE），当存在残疾风险时，如广泛性或系统性硬斑病和进行性线状硬皮病，需要对局限性硬皮病进行全身性治疗。在青少年系统性硬化症中，严重程度评分 J4S 的引入使患者在日常实践中的评估标准化，并允许采用更具针对性的治疗方法。由于 JSSc 的罕见性，迄今为止尚无临床试验，因此治疗基于成人的经验。青少年硬皮病的最新建议是标准化治疗方法的重要工具，证实了甲氨蝶呤的作用，并为严重或难治性病例的有效实验治疗方法（如霉酚酸酯和生物制剂）开辟了新的窗口。
