肺高血压在连接组织疾病,除了系统性硬化症
Brandon Budhram1, Jason Weatherald2, Marc Humbert3
1Division of Respirology, Department of Medicine, University of Calgary, Calgary, AB, Canada.
Seminars in respiratory and critical care medicine
|March 18, 2024
概括
肺高血压 (PH) 在连接组织疾病 (CTD) 其他系统性硬化症 (SSc) 需要不同的临床方法. 本综述详细介绍了与非SScCTD相关的PH的诊断和治疗.
科学领域:
- 类风湿病学 类风湿病学
- 心脏病学 心脏病学
- 肺部病理学 肺部病理学
背景情况:
- 肺高血压 (PH) 是连接组织疾病 (CTD) 的严重并发症.
- 系统性硬化症 (SSc) 是与PH相关的最常见的CTD,但非SScCTD存在独特的挑战.
- 非SScCTD (如狼,Sjögren) 的PH具有不同的流行病学,病理生理学和治疗需求.
研究的目的:
- 为临床医生提供评估和管理非SScCTD患者的PH的实用指南.
- 要突出非SScCTD中PH的独特特征,与SSc相关的PH相比.
- 审查当前的诊断和治疗策略,确定未来研究领域.
主要方法:
- 对非SScCTDs的PH现有文献的审查.
- 对病理生理机制的分析,包括遗传,免疫和内皮因素.
- 对诊断工具的检查:生物标志物,成像和程序.
- 评估医疗,干预和手术治疗选择.
主要成果:
- 非SScCTD中的PH源于复杂的相互作用和多样化的机制 (组1-5PH).
- 早期诊断至关重要,并得到特定生物标志物和诊断程序的支持.
- 治疗策略必须考虑CTD特定因素以获得最佳结果.
结论:
- 非SScCTD中的PH是一种独特的临床实体,需要量身定制的管理.
- 需要进一步的研究,以解决目前的理解和治疗方面的差距.
- 为了改善这些患者的预后,必须采用多学科的方法.
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