韩国青少年系统性硬化症的临床特征:为期31年的单中心研究
Ji Eun Jeong1, Seong Heon Kim1
1Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.
Journal of rheumatic diseases
|December 22, 2023
概括
这项研究分析了韩国青少年系统性硬化症 (JSSc),发现雷诺症.
科学领域:
- 儿科风湿病学 儿科风湿病学
- 自免疫性疾病 自免疫性疾病
- 皮肤病学 皮肤病学
背景情况:
- 青少年系统性硬化症 (JSSc) 是一种罕见的自身免疫性疾病,影响儿童.
- 了解其临床表现和进展对于早期诊断和管理至关重要.
研究的目的:
- 描述韩国JSSc患者的临床和实验室特征.
- 评估单中心队列中的治疗结果和预后.
主要方法:
- 从1992年到2023年,对13名患有JSSc (发病时间小于16年) 的患者进行了回顾性分析.
- 根据临时分类标准进行纳入,但不包括局部性硬皮病.
- 数据收集包括临床迹象,症状,自身抗体和器官参与.
主要成果:
- 临近皮肤硬化症 (100%),雷诺现象 (84.6%) 和硬化症 (69.2%) 在诊断时很常见.
- RP是最常见的先前症状 (76.9%);近接性皮肤硬化症通常最初错过 (38.5%).
- 在所有患者中观察到阳性抗核抗体 (ANA). 间歇性肺病 (ILD) 在5/13例中在诊断时被检测到 (2例无症状),并在随访期间在3例中发展. 脏和心脏疾病发生在每位1名患者身上;没有报告死亡.
结论:
- 这是韩国JSSc的第一个描述性分析.
- 在患有RP和阳性ANA的儿童中,即使没有明显的皮肤硬化症,对JSSc的临床怀疑至关重要.
- 肺功能测试 (PFTs) 建议用于早期ILD检测,无论胸部CT发现或症状如何.
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