系统性硬化症 性硬化症:重新评估的时间
Anastasios Makris1, Alexandros Panagiotopoulos1, Oliver Distler2
1A. Makris, MD, A. Panagiotopoulos, MD, P.P. Sfikakis, MD, PhD, First Department of Propaedeutic and Internal Medicine, National and Kapodistrian University of Athens, Medical School, Athens, Greece.
The Journal of rheumatology
|July 1, 2024
概括
系统性硬化无硬化症 (ssSSc) 影响近10%的患者,经常出现严重的器官干扰. 早期分类标准可能导致诊断不足,需要更新准则以准确管理.
科学领域:
- 类风湿病学 类风湿病学
- 内部医学 内部医学
- 临床免疫学临床免疫学
背景情况:
- 系统性硬化无硬化症 (ssSSc) 是一种独特的系统性硬化症 (SSc) 的子集,其特点是缺乏皮肤纤维化.
- 根据2013年ACR/EULAR标准,根据2013年ACR/EULAR标准,SSc的诊断可以在没有皮肤加厚的情况下发生,即使它后来发展.
- 一个致命的SSSSc病例引起了对现有数据的分析.
研究的目的:
- 根据已发表的文献分析系统性硬化无硬化症 (ssSSc) 的患病率和严重程度.
- 综合SSSSc.的临床表现和结果的数据.
- 确定ssSSc诊断和分类中的挑战.
主要方法:
- 进行了系统的文献审查和SSc队列的定性合成.
- 分析了35项研究的数据,包括25455名SSc患者,1976年至2023年间发表的数据.
- 流行率,重新分类率,器官参与和生存数据被提取和合成.
主要成果:
- SSSSc的平均患病率约为10%,在研究中具有显著的变化.
- 在随访期间,在0-28%的病例中,SSSSc被重新分类为有限或扩散的皮肤SSc.
- 在sssssc患者中观察到高比例的间歇性肺部疾病 (46%),肺动脉高血压 (15%),心脏扩张功能障碍 (26.5%).
结论:
- 关于SSSSc患病率,临床特征和预后的公布数据显示出相当大的差异,归因于诊断不足和错误分类.
- 当前的分类标准可能无法完全捕捉ssSSc.的频谱.
- 建议更新ssSSc的分类标准,考虑到疾病发病时间,以改善患者管理.
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