硬化肌病患者的临床表现谱和皮肤和肺部卷入之间的相关性
Jyotsna Oak1, Ojas Unavane2, Rupali Mathur2
1Head of the Department, General Medicine, and Consultant in Rheumatology, Department of Rheumatology, Mumbai, Maharashtra, India, Corresponding Author.
The Journal of the Association of Physicians of India
|March 15, 2025
概括
系统性硬化症 (SSc) 患者经常会出现严重的肺部并发症,如肺高血压和间歇性肺病,无论疾病的亚型如何. 通过全面评估进行早期检测对于更好的结果至关重要.
科学领域:
- 类风湿病学 类风湿病学
- 肺部病理学 肺部病理学
- 皮肤病学 皮肤病学
背景情况:
- 系统性硬化症 (SSc) 是一种复杂的自身免疫性疾病,其特征是皮肤和内部器官纤维化.
- 肺部和皮肤表现显著影响患者的预后和生活质量.
研究的目的:
- 描述SSc患者的各种症状和症状的特征.
- 调查SSc.肺部和皮肤参与之间的相关性.
主要方法:
- 综合性临床评估,包括病史和体检.
- 诊断调查:血液样本,ESR,和肝功能测试,心电图,HRCT和肺功能测试.
- 修改的罗丹皮肤评分 (mRSS) 用于评估皮肤卷入.
主要成果:
- 在71.2%的患者中观察到分散的皮肤SSc (dcSSc),在28.8%的患者中观察到有限的皮肤SSc (lcSSc).
- 常见的症状包括雷诺现象 (92.3%),皮肤收紧 (88.4%) 和硬化症 (88.4%).
- 肺部干扰:59.6%的患者患有间歇性肺病 (ILD),13.5%的患者患有肺高血压 (PH);mRSS和肺部干扰之间存在显著的相关性 (p < 0.05).
结论:
- 严重的系统性并发症,如ILD和PH可以发生在SSc患者,无论疾病的持续时间或亚型.
- 症状可能并不总是与疾病进展相关,需要彻底评估.
- 通过症状和诊断测试 (PFT,ECHO,HRCT) 及早发现对于及时治疗和改善预后至关重要;mRSS对监测有价值.
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