硬皮病临床试验联合损伤指数在青少年系统性硬化症患者中的应用。

IF 2.8 3区 医学 Q1 PEDIATRICS
Caihui Zhang, Changyan Wang, Sihao Gao, Mingsheng Ma, Wei Wang, Tianyu Zhang, Yu Zhang, Xiaoyan Tang, Zhuo Li, Zhixing Sun, Lin Wang, Hongzhong Jin, Xiaofeng Zeng, Hongmei Song
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引用次数: 0

摘要

背景:青少年系统性硬化症(jSSc)可导致永久性和不可逆的解剖或生理功能障碍。硬皮病临床试验联盟损伤指数(SCTC-DI)已在成人患者中得到应用和验证,可以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探索。方法:收集2012年1月至2024年1月北京协和医院收治的jSSc患者的临床资料、实验室检查结果及预后信息。记录SCTC-DI与青少年系统性硬化症严重程度评分(J4S)的差异并进行比较。此外,我们比较了jSSc和成人系统性硬化症(SSc)患者的SCTC-DI。结果:共纳入64例jSSc患者。小关节挛缩、指尖溃疡和肺间质性疾病是常见的表现。与成人SSc患者相比,jSSc患者胃肠道和泌尿系统受累的发生率较低。基线J4S水平与随访时SCTC-DI水平显著相关。基线SCTC-DI评分越高,器官损伤进展越严重(P = 0.001)。结论:成人SSc患者与青少年SSc患者在临床表现上存在差异。SCTC-DI可用于JSSc患者,建议JSSc患者定期进行J4S和SCTC-DI评估。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Application of the Scleroderma Clinical Trials Consortium Damage Index in patients with juvenile systemic sclerosis.

Background: 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.

Methods: 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.

Results: 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).

Conclusion: 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.

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来源期刊
Pediatric Rheumatology
Pediatric Rheumatology PEDIATRICS-RHEUMATOLOGY
CiteScore
4.10
自引率
8.00%
发文量
95
审稿时长
>12 weeks
期刊介绍: Pediatric Rheumatology is an open access, peer-reviewed, online journal encompassing all aspects of clinical and basic research related to pediatric rheumatology and allied subjects. The journal’s scope of diseases and syndromes include musculoskeletal pain syndromes, rheumatic fever and post-streptococcal syndromes, juvenile idiopathic arthritis, systemic lupus erythematosus, juvenile dermatomyositis, local and systemic scleroderma, Kawasaki disease, Henoch-Schonlein purpura and other vasculitides, sarcoidosis, inherited musculoskeletal syndromes, autoinflammatory syndromes, and others.
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