与连接组织疾病相关的肺动脉高血压 (CTD-PAH):最近和先进的数据
Benjamin Thoreau1, Luc Mouthon1
1Department of Internal Medicine, Referral Center for Rare Autoimmune and Systemic Diseases, AP-HP.Centre, Université Paris Cité, Hôpital Cochin, 27, rue du Faubourg Saint-Jacques, 75679 Cedex 14 Paris, France; Université Paris Cité, F-75006 Paris, France; INSERM U1016, Cochin Institute, CNRS UMR 8104, Université Paris Cité, Paris, France.
连接组织疾病 (CTD-PAH) 中的肺动脉高血压 (PAH) 有显著的影响. 本综述澄清了CTD-PAH定义,病原和治疗,以改善患者的治疗结果.
科学领域:
- 心脏病学和风湿病学.
- 罕见疾病 罕见疾病
- 肺高血压是什么意思 肺高血压
背景情况:
- 肺动脉高血压 (PAH) 是一种严重的疾病,影响病率和死亡率.
- 连接组织疾病 (CTD-PAH) 是二次PAH的一个重要原因.
- 系统性硬化症 (SSc),系统性红斑狼 (SLE) 和混合结缔组织疾病 (MCTD) 是CTD-PAH关键关联,具有地理差异.
研究的目的:
- 审查关于更新的PAH定义和分类的最新文献.
- 阐明CTD-PAH的发病,流行病学,检测,预后和治疗.
- 为了澄清CTD-PAH的特定特征,尽管最近的治疗进展.
主要方法:
- 对最近的科学文献进行全面的审查.
- 对有关PAH分类,定义和查的数据进行分析.
- 对CTD-PAH的流行病学,病原遗传学和治疗方面的评估.
主要成果:
- 最近在PAH分类,风险分层和治疗策略方面取得了进展.
- 需要进一步澄清CTD-PAH的特点,包括SSc,SLE和MCTD等病因驱动因素.
- 在CTD-PAH患病率 (例如,西方的SSc,亚洲的SLE/MCTD) 中存在地理差异.
结论:
- 尽管取得了进展,但对CTD-PAH特异性的清晰理解仍然不完整.
- 需要进一步的研究来完善CTD-PAH的诊断,风险评估和向治疗.
- 本综述综合了当前的知识,以指导CTD-PAH的未来临床实践和研究.
更多相关视频
08:08Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
Published on: May 11, 2015
07:02A Large Animal Model for Pulmonary Hypertension and Right Ventricular Failure: Left Pulmonary Artery Ligation and Progressive Main Pulmonary Artery Banding in Sheep
Published on: July 15, 2021
相关概念视频
Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...
Pulmonary Hypertension: Classification and Pathogenesis
There are various classifications for PH, each relating to different underlying causes and also...
Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors
Among the PDE5 inhibitors, sildenafil (Revatio) stands out as a competitive and selective inhibitor. It operates by elevating cellular levels of cGMP and augmenting signaling through the cGMP-PKG pathway, promoting vasodilation. Upon oral...
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue,...
COPD: Pathogenesis and Clinical Features
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
