Zhang Caihui, Wang Changyan, Gao Sihao, Ma Mingsheng, Wang Wei, Zhang Tianyu, Zhang Yu, Tang Xiaoyan, Li Zhuo, Sun Zhixing, Wang Lin, Jin Hongzhong, Zeng Xiaofeng, Song Hongmei
Department of Pediatrics, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, No. 1 Shuaifuyuan Wangfujing Dongcheng District, Beijing, 100730, China.
Department of Dermatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.
Pediatr Rheumatol Online J. 2025 May 14;23(1):53. doi: 10.1186/s12969-025-01110-6.
Juvenile systemic sclerosis (jSSc) can lead to permanent and irreversible anatomical or physiological dysfunction. The Scleroderma Clinical Trials Consortium-Damage Index (SCTC-DI), which has been employed and validated in adult patients, can quantify organ damage and predict mortality and morbidity. However, its application in paediatric patients remains unexplored.
Clinical data, laboratory results, and prognostic information were collected for patients with jSSc at Peking Union Medical College Hospital (PUMCH) from January 2012 and January 2024. Differences between the SCTC-DI and the juvenile systemic sclerosis severity score (J4S) were recorded and compared. Furthermore, we compared the SCTC-DI between jSSc and adult systemic sclerosis (SSc) patients.
A total of 64 jSSc patients were included. Facet joint contractures, fingertip ulcers and interstitial lung disease are common manifestations. Compared with adult SSc patients, jSSc patients had a lower incidence of gastrointestinal and urinary system involvement. The baseline J4S levels were significantly correlated with SCTC-DI levels at follow-up. A higher baseline SCTC-DI score was associated with a greater progression of organ damage (P = 0.001).
There are differences in clinical presentations between adult SSc patients and jSSc patients. The SCTC-DI can be applied to JSSc patients, and it is recommended that JSSc patients undergo regular evaluations of the J4S as well as the SCTC-DI.
青少年系统性硬化症（jSSc）可导致永久性和不可逆的解剖学或生理学功能障碍。硬皮病临床试验联盟损伤指数（SCTC-DI）已在成年患者中应用并得到验证，可量化器官损伤并预测死亡率和发病率。然而，其在儿科患者中的应用仍未得到探索。
收集了2012年1月至2024年1月在北京协和医院（PUMCH）就诊的jSSc患者的临床数据、实验室检查结果和预后信息。记录并比较了SCTC-DI与青少年系统性硬化症严重程度评分（J4S）之间的差异。此外，我们还比较了jSSc患者与成年系统性硬化症（SSc）患者之间的SCTC-DI。
共纳入64例jSSc患者。小关节挛缩、指尖溃疡和间质性肺疾病是常见表现。与成年SSc患者相比，jSSc患者胃肠道和泌尿系统受累的发生率较低。随访时基线J4S水平与SCTC-DI水平显著相关。基线SCTC-DI评分越高，器官损伤进展越大（P = 0.001）。
成年SSc患者和jSSc患者的临床表现存在差异。SCTC-DI可应用于jSSc患者，建议jSSc患者定期评估J4S以及SCTC-DI。
