肺高血压的预测因素和预后使系统性硬化症中间歇性肺病复杂化
Kathleen Morrisroe1,2, Dylan Hansen2, Wendy Stevens2
1Department of Medicine, The University of Melbourne at St Vincent's Hospital, Melbourne, Victoria, Australia.
系统性硬化症患者患有肺高血压和间歇性肺病的生存率很低. 血管扩张剂治疗可能会改善这些患者的症状和生活质量,但不能改善这些患者的生存率.
科学领域:
- 类风湿病学 类风湿病学
- 肺部病理学 肺部病理学
- 心脏病学 心脏病学
背景情况:
- 系统性硬化症 (SSc) 是一种复杂的自身免疫性疾病.
- 肺高血压 (PH) 和间歇性肺病 (ILD) 是SSc的严重并发症.
- 在SSc中识别和描述并发的PH和ILD对于管理至关重要.
研究的目的:
- 为了确定SSc患者与同时PH和ILD.
- 为了确定这个队列中的疾病严重程度,治疗策略和生存结果.
- 为了比较PH-ILD和1组PAH的SSc患者之间的结果.
主要方法:
- 来自澳大利亚结核病队列研究 (ASCS) 的连续SSc患者的分析.
- 通过右心导管检查证实PH的诊断.
- 使用了后勤回归和卡普兰-梅尔生存分析.
主要成果:
- 8.7%的SSc患者患有事件PH;43.9%的人患有并发性ILD (PH-ILD).
- 在PH-ILD患者中有40.3%存在广泛的ILD.
- 血管扩张疗法在PH-ILD和1组PAH中同样使用和耐受,但没有改善PH-ILD的生存率,尽管它改善了症状和生活质量.
结论:
- 在SSc-PH中同时出现ILD与更差的生存率有关.
- 广泛ILD的SSc-PH的血管扩张疗法耐受性很好,可以提高生活质量,尽管没有改善生存率.
- 对于SSc-PH患者来说,即使使用血管扩展剂治疗,生存率仍然很差.
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