Häfner R, Truckenbrodt H
Klin Padiatr. 1986 Sep-Oct;198(5):401-7. doi: 10.1055/s-2008-1033897.
The course of disease in 187 patients with systemic juvenile chronic arthritis was documented in a retrospective study 2-20 years after onset. Despite differences in joint involvement in the first 6 months of the disease, most of the patients subsequently developed a polyarthritis leading to severely impaired function in approximately 50% of them. Advanced degenerative changes identified by roentgenography were found in particular in the wrist and hip joints. There was an overall regression in the frequency of the extra-articular symptoms and the humoral pathologic activity. Fifteen years after the onset of disease only 30% of the patients still had systemic signs, and humoral activity was found in less than 50%. The mortality rate of 14% was primarily due to infections and kidney failure in amyloidosis. The course varies considerably from child to child. It has not so far been possible to differentiate prognostic factors for different subgroups.
一项回顾性研究记录了187例系统性幼年慢性关节炎患者发病2至20年后的疾病进程。尽管疾病最初6个月关节受累情况存在差异，但大多数患者随后发展为多关节炎，约50%的患者功能严重受损。通过X线检查发现的晚期退行性改变尤其见于腕关节和髋关节。关节外症状的频率和体液病理活动总体呈消退趋势。疾病发作15年后，仅30%的患者仍有全身症状，且体液活动者不到50%。14%的死亡率主要归因于感染和淀粉样变性所致的肾衰竭。疾病进程在儿童之间差异很大。迄今为止，尚未能够区分不同亚组的预后因素。
