Sarcoidosis 与肺高血压相关的肺高血压
Alexander Liu1, Laura C Price1, Rakesh Sharma1
1Royal Brompton Hospital, Part of Guy's and St. Thomas' NHS Foundation Trust, London SW3 6NP, UK.
Biomedicines
|January 23, 2024
概括
麻症相关肺高血压 (SAPH) 影响2.9-20%的患者,早期诊断具有挑战性. 预后与6分钟步行测试和肺部疾病严重程度有关.
科学领域:
- 肺部医学 肺部医学
- 心脏病学 心脏病学
- 类风湿病学 类风湿病学
背景情况:
- 麻症相关肺高血压 (SAPH) 显著增加了麻症患者的发病率和死亡率.
- SAPH的患病率在2.9%至20%之间,多因素导致,包括肺部疾病,心脏功能障碍和外部压缩.
- 正确的SAPH早期诊断受到当前非侵入性查策略的局限性阻碍.
研究的目的:
- 审查当前对沙尔科毒症相关的肺高血压的理解.
- 突出SAPH的诊断挑战和预后指标.
- 讨论SAPH目前和潜在的未来管理策略.
主要方法:
- 审查现有文献和注册表的结果.
- 对促进SAPH发展和预后的因素的分析.
- 评估诊断工具,包括胸前心脏回声和右心脏导管.
- 评估治疗方式和肺移植的作用.
主要成果:
- 6分钟步行测试 (6MWT) 在SAPH患者中显示出显著的预后价值.
- 晚期肺部疾病,以降低的DLCO (预测<35%) 表示,与毛囊前SAPH中减少的无移植存活率相关.
- 在疑似SAPH的情况下,胸外回声心脏成像是推用于右心脏导管 (RHC) 的查工具.
结论:
- 有效的SAPH管理需要解决潜在的机制,肺血管扩展剂在特定的表型中是有益的.
- 肺移植是SAPH末期的高风险选择.
- 进一步的多中心随机对照试验对于推进SAPH疗法和改善患者治疗结果至关重要.
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