连接组织疾病相关的肺高血压:综合性综述
Vikramjit Khangoora1, Elana J Bernstein2, Christopher S King1
1Advanced Lung Disease and Transplant Program Inova Fairfax Hospital Falls Church Virginia USA.
Pulmonary circulation
|December 13, 2023
概括
连接组织疾病 (CTD) 可以导致肺高血压 (PH),特别是在全身性硬化症 (SSc) 中. 早期查和血液动力学评估对于管理CTD-PH至关重要,尽管结果仍然比特异性PAH差.
科学领域:
- 心脏病学 心脏病学
- 肺部病理学 肺部病理学
- 类风湿病学 类风湿病学
背景情况:
- 连接组织疾病 (CTD) 与各种形式的肺高血压 (PH) 有关.
- 肺动脉高血压 (PAH) 在CTD的患病率范围,在全身性硬化症 (SSc) 中最高,为8-12%.
研究的目的:
- 审查CTD和PH之间的关联.
- 为突出查,诊断和管理策略的CTD相关PH.
- 讨论CTD-PAH与异常性PAH相比的较差结果.
主要方法:
- 审查当前的文献和指导方针.
- 强调右心脏导管治疗用于血液动力学分析.
- 基于肺血管病变现型的治疗策略的讨论.
主要成果:
- 建议在SSc和相关CTD中进行PAH的常规查.
- 准确的诊断需要彻底的评估,包括右心脏导管.
- CTD-PAH的结果低于异常性PAH,特别是在SSc-PAH中.
结论:
- 对CTD-PAH的管理通常遵循异常性PAH指南.
- 肺外表现和并发症有助于更糟糕的结果.
- 建议提前转移肺移植并进一步研究病原体.
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