系统性硬化症患者的皮肤表现,临床特征和预后:来自国际EUSTAR数据库的数据
Alain Lescoat1,2,3,4, Suiyuan Huang3,4, Patricia E Carreira5
1University of Rennes, CHU Rennes, Inserm, EHESP, Irset (Institut de Recherche en Santé, Environnement et Travail) - UMR_S 1085, Rennes, France.
JAMA dermatology
|June 28, 2023
概括
与其他SSc子集相比,全身性硬化无硬化症 (ssSSc) 患者的存活率更高,手指和指的患病率更低. 然而,间歇性肺病仍然很常见,皮肤上的发现,如telangiectasias可能表明心脏功能障碍.
科学领域:
- 类风湿病学 类风湿病学
- 皮肤病学 皮肤病学
- 内部医学 内部医学
背景情况:
- 系统性硬化症 (SSc) 性硬化症 (ssSSc) 是一种由皮肤纤维化不存在的特征.
- 天然史和ssSSc的特定皮肤表现并未得到充分了解.
研究的目的:
- 为了比较SSSSc患者的临床表型,限制皮肤SSc (lcSSc) 和扩散皮肤SSc (dcSSc).
- 分析ssSSc患者的生存率和皮肤表现.
主要方法:
- 一项使用国际EUSTAR数据库的纵向观察队列研究.
- 包括符合SSc分类标准的患者,至少进行一次随访.
- ssSSc通过修改的罗丹皮肤评分 (mRSS) 定义为0和在所有访问中没有硬化症.
主要成果:
- 在4263名患者中,376名 (8.8%) 患有SSSSc. 与lcSSc和dcSSc相比,ssSSc患者的指部和指部发的发生率较低.
- 间歇性肺病的患病率在ssSSc和lcSSc中类似,但在dcSSc中更高.
- 在ssSSc中的皮肤端膜切除与腹功能障碍有关. 反Scl-70阳性预测了ssSSc.中的皮肤纤维化发病.
- 在15年后,SSSSc (92.4%) 的生存率高于lcSSc (69.4%) 和dcSSc (55.5%).
结论:
- ssSSc患者表现出明显的临床特征,生存率更好,但患间歇性肺病的风险很大.
- 皮肤病学家必须认识到ssSSc中的皮肤发现可能与内部器官功能障碍相关,例如与telangiectasias相关的透气心脏问题.
- 由于其涉及内部器官的普遍性和独特的预后指标,SSSSc值得注意.
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