Foeldvari Ivan
Hamburg Centre for Children and Youth Rheumatology, Kompetenzzentrum für Sklerodermie im Kindes und Jugendalter, am Klinikum Eilbek, Hamburg, Germany.
Curr Opin Rheumatol. 2008 Sep;20(5):608-12. doi: 10.1097/BOR.0b013e3283103cfd.
Aim of the review is to summarize the current knowledge about clinical presentation and prognosis of patients with juvenile onset systemic sclerosis and compare it with that of those with adult onset systemic sclerosis.
Juvenile onset systemic sclerosis and adult onset systemic sclerosis have a different subtype and organ involvement pattern of the juvenile onset patients in the pediatric age is compared with that of adult patients. The juvenile onset patients have 90% diffuse subset, despite a 5-year survival of around 90%. The organ involvement pattern in both the groups becomes equal over the disease duration, but renal involvement is rare. The subset pattern equalizes over time too; the adult-aged juvenile onset patients have the same disease subset distribution, with around 40% diffuse subset patients, as the young adult onset patients, and this presents a survival bias presumably. The survival even after 20-year disease course seems more favourable for the juvenile onset patients.
Currently we are looking at two time windows: one at the first 5 years of the disease and the second at 20 years after the onset. We do not have the data on why and when the subset distribution changes occur.
本综述旨在总结目前关于青少年起病系统性硬化症患者临床表现和预后的知识，并将其与成人起病系统性硬化症患者的情况进行比较。
青少年起病系统性硬化症和成人起病系统性硬化症具有不同的亚型，且儿科年龄的青少年起病患者与成人患者的器官受累模式有所不同。青少年起病患者90%为弥漫型亚型，尽管其5年生存率约为90%。两组患者的器官受累模式在疾病过程中会趋于相同，但肾脏受累罕见。亚型模式也会随时间趋于一致；成年期的青少年起病患者与青年成人起病患者具有相同的疾病亚型分布，约40%为弥漫型亚型患者，这可能存在生存偏差。即使在病程20年后，青少年起病患者的生存情况似乎更有利。
目前我们关注两个时间窗口：一个是疾病的前5年，另一个是发病后20年。我们没有关于亚型分布变化为何以及何时发生的数据。
