Abstract: Juvenile systemic sclerosis （JSSc） is a chronic autoimmune disease with its pathogenesis not fully understood， and both genetic and environmental factors are involved in the development of the disease. Early diagnosis is essential， and early intervention is related to good prognosis. During diagnosis and treatment， the patient should be systematically evaluated regularly. Clinical evaluation tools such as juvenile systemic sclrosis severity score（J4S） scoring and modified Rodnan skin score（mRSS）scoring have been widely used in clinical practice. Skin ultrasound and nail fold microcircul-ation can evaluate the disease to a certain extent. A combina-tion of glucocorticoids， methotrexate， cyclophosph-amide， or mycop-henolate mofetil can be considered depen-ding on the severity of the organ involvement. In cases unresponsive to immunosuppressive agents or glucocorticoids， biological agents such as tocilizumab，rituximab or hematopo-ietic stem cell transplantation can be selected. As compared with adults， JSSc have overall better outcomes and lower mortality.

Key words: systemic sclerosis, pathogenesis, child, diagnosis, treatment

摘要： 儿童系统性硬化症是一种罕见的自身免疫性疾病，其发病机制尚未完全明确，遗传与环境因素均参与该疾病的发生发展。早期诊断至关重要，早期干预与良好的预后相关，诊疗过程中应定期系统评估。系统性硬化症严重程度评分（J4S评分）、改良Rodnan皮肤评分（mRSS评分）等临床评估工具已广泛应用于临床，皮肤超声、甲襞微循环等可在一定程度上评估病情，治疗方案包括糖皮质激素、甲氨蝶呤、吗替麦考酚酯及环磷酰胺等，若效果欠佳，可选择托珠单抗、利妥昔单抗等生物制剂或造血干细胞移植。儿童系统性硬化症临床表现与成人类似，但预后相对较好，病死率较低。

关键词: 系统性硬化症, 发病机制, 儿童, 诊断, 治疗