Sarcoidosis 相关的肺高血压
Dominique Israël-Biet1, Jean Pastré2, Hilario Nunes3,4
1Université Paris Cité, 75006 Paris, France.
Journal of clinical medicine
|April 13, 2024
概括
麻症相关的肺高血压 (SAPH) 是一种严重的并发症,影响3-20%的麻症患者,特别是那些晚期肺病患者. 了解其机制是个性化治疗策略的关键.
科学领域:
- 肺部病理学 肺部病理学
- 心脏病学 心脏病学
- 类风湿病学 类风湿病学
背景情况:
- 麻症相关的肺高血压 (SAPH) 是麻症的一种严重并发症.
- 它显著影响患者的发病率和死亡率.
- 发病率估计在3-20%的沙尔科病患者中,尤其是晚期纤维化肺病患者.
研究的目的:
- 审查当前对 Sarcoidosis 相关的肺高血压的理解.
- 突出诊断方法和理解治疗的潜在机制的重要性.
主要方法:
- 审查当前的文献和临床指南.
- 讨论诊断工具,包括心声回声和右心导管.
- 强调SAPH机制的多因素性质.
主要成果:
- 已经更新了SAPH定义 (平均肺动脉压>20 mmHg).
- 右心脏导管是诊断的黄金标准.
- 心声图是主要的非侵入性查工具.
- SAPH的机制是复杂的,需要进一步阐明.
结论:
- 沙性硬化症 (SAPH) 是沙性硬化症的一个关键并发症,具有显著的死亡影响.
- 准确的诊断依赖于非侵入性查和侵入性确认的组合.
- 个性化治疗需要对每个患者的具体潜在机制有充分的了解.
相关概念视频
Pulmonary Hypertension: Classification and Pathogenesis
173
Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
There are various classifications for PH, each relating to different underlying causes and also...
There are various classifications for PH, each relating to different underlying causes and also...
173
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
156
Endothelins (ETs) are potent vasoactive peptides critical in the human body's various physiological and pathological processes. One of the most promising therapeutic strategies for treating pulmonary arterial hypertension (PAH) involves counteracting the effects of these endothelins using a class of drugs known as endothelin receptor antagonists.
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
156
Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors
150
Phosphodiesterase 5 (PDE5) inhibitors are potent enzymes that function to hydrolyze cyclic nucleotides to their corresponding 5' monophosphates. Their unique biochemical properties have been applied in treating Pulmonary Arterial Hypertension (PAH).
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...
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...
150
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure
199
Oxygen therapy has emerged as a significant tool in enhancing the quality of life for patients suffering from pulmonary arterial hypertension (PAH). While this therapy has principally been studied on patients with significant hypoxemia, this therapeutic approach helps prevent potential organ damage and can be administered in the comfort of one's home.
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue,...
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue,...
199
Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers
160
Receptor tyrosine kinase inhibitors (TKIs) and calcium channel blockers (CCBs) are two critical categories of drugs employed in the treatment of pulmonary artery hypertension (PAH). PAH is a disease that causes high blood pressure in the pulmonary arteries, resulting in chest pain, fatigue, and shortness of breath.
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...
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...
160
Pulmonary Tuberculosis II
228
Tuberculosis, or TB, is a bacterial infectious disease caused by Mycobacterium tuberculosis. While its primary impact is on the lungs, leading to pulmonary tuberculosis, it can also affect various other organs, a condition referred to as extrapulmonary tuberculosis.
Here is a detailed explanation of its pathophysiology:
Transmission: The process begins when a person inhales droplet nuclei containing M. tuberculosis. These are typically released into the air when an individual with pulmonary or...
Here is a detailed explanation of its pathophysiology:
Transmission: The process begins when a person inhales droplet nuclei containing M. tuberculosis. These are typically released into the air when an individual with pulmonary or...
228


