Hamburger Zentrum für Kinder- und Jugendrheumatologie, Kompetenz Zentrum für Sklerodermie im Kindesalter, Kompetenz Zentrum für autoimmune Uveitis im Kindesalter, Am Klinikum Eilbek, Dehnhaide 120, D-22081 Hamburg, Germany.
Rheumatology (Oxford). 2012 Oct;51(10):1832-7. doi: 10.1093/rheumatology/kes144. Epub 2012 Jun 22.
The aim of the present study was to explore the long-term outcome and clinical characteristics of adult patients with juvenile onset in the EULAR Scleroderma Trials and Research (EUSTAR) cohort and compare them with adult patients with onset between 20 and 40 years of age.
From the EUSTAR SSc cohort two patient groups were analysed: patients with juvenile SSc (jSSc) who are adults at present, and patients diagnosed between the age of 20 and 40 years (aSSc). Demographic data of the patients, organ involvement and outcome of the disease were examined using the Minimal Essential Data Set database system.
From 5000 patients in the EUSTAR cohort, 60 patients (1.2%) with jSSc and 910 patients (18%) with aSSc were selected according the inclusion criteria. In the jSSc group, the mean age of disease onset was 12.4 years (range 2-15.9 years), and in the aSSc group, the mean age was 32 years (range 20-40 years). Disease subsets were similar. The antibody profile was also comparable except for ACAs, which were positive in 5% of the jSSc group and 26.9% of the aSSc group (P<0.005). Organ involvement (lung, kidney, joint, muscle and heart) was similar in the two groups of patients at the time of the last follow-up.
The subset distribution in the jSSc and aSSc cohorts was found to be similar. Only the frequency of ACAs was significantly lower in the jSSc, which supports the hypothesis that the SSc patients with paediatric onset in the adult cohort may represent a distinct subgroup of the complete cohort of paediatric patients.
本研究旨在探讨 EULAR 硬皮病试验和研究（EUSTAR）队列中青少年发病的成年患者的长期预后和临床特征，并将其与 20-40 岁发病的成年患者进行比较。
从 EUSTAR SSc 队列中分析了两组患者：目前为成年人的青少年硬皮病（jSSc）患者和 20-40 岁（aSSc）诊断的患者。使用最小基本数据集中的数据库系统检查患者的人口统计学数据、器官受累和疾病结果。
从 EUSTAR 队列的 5000 名患者中，根据纳入标准选择了 60 名（1.2%）jSSc 患者和 910 名（18%）aSSc 患者。在 jSSc 组中，疾病发病的平均年龄为 12.4 岁（范围 2-15.9 岁），在 aSSc 组中，平均年龄为 32 岁（范围 20-40 岁）。疾病亚型相似。除 ACA 外，抗体谱也相似，jSSc 组阳性率为 5%，aSSc 组阳性率为 26.9%（P<0.005）。在最后一次随访时，两组患者的器官受累（肺、肾、关节、肌肉和心脏）相似。
jSSc 和 aSSc 队列的亚组分布相似。仅在 jSSc 中 ACA 的频率明显较低，这支持了在成年队列中儿童发病的 SSc 患者可能代表完整儿童患者队列中的一个不同亚组的假说。
