■青少年系统性硬化症（jSSc）是一种孤儿疾病，患病率为1,000,000名儿童中的3名。目前只有一个关于皮肤的共识治疗指南，jSSc的肺和血管受累，JSScSHARE（欧洲儿科风湿病单集线器和接入点）倡议，这是基于截至2014年的数据。因此,这些指南的更新，凭借最近的文学和专家经验，并且需要将指导扩展到疾病的更多方面。
■审查了治疗方案，并为jSSc的大多数方面提供了意见，包括一般管理，一些不同于成人系统性硬化症，比如使用皮质类固醇，和特定的器官受累，如皮肤，肌肉骨骼，肺,胃肠病学等.
■我们建议以早期治疗为目标的治疗策略，以防止jSSc中的累积疾病损害。结论来自专家意见和现有文献，主要基于成人系统性硬化症（aSSc），鉴于共同的病理生理学，对aSSc研究结果的推断被认为是合理的。
UNASSIGNED: Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1,000,000 children. Currently there is only one
consensus treatment
guideline concerning skin, pulmonary and vascular involvement for jSSc, the jSSc SHARE (Single Hub and Access point for pediatric Rheumatology in Europe) initiative, which was based on data procured up to 2014. Therefore, an update of these
guidelines, with a more recent literature and expert experience, and extension of the guidance to more aspects of the disease is needed.
UNASSIGNED: Treatment options were reviewed, and opinions were provided for most facets of jSSc including general management, some of which differs from adult systemic sclerosis, such as the use of corticosteroids, and specific organ involvement, such as skin, musculoskeletal, pulmonary, and gastroenterology.
UNASSIGNED: We are suggesting the treat to target strategy to treat early to prevent cumulative disease damage in jSSc. Conclusions are derived from both expert opinion and available literature, which is mostly based on adult systemic sclerosis (aSSc), given shared pathophysiology, extrapolation of results from aSSc studies was judged reasonable.
