强迫生命能力作为系统性硬化症相关肺高血压的生存预测因素
Claudia Iannone1,2, Stefano Stano3, Maria Rosa Pellico1,2
1Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy.
Therapeutic advances in musculoskeletal disease
|January 22, 2026
概括
对于患有肺高血压和全身性硬化症 (SSc-PH) 的患者来说,强迫性生命能力 (FVC) 低于70%是预后不良的关键指标,无论间歇性肺病 (ILD) 的程度如何. 常规的肺功能监测对于风险分层至关重要.
科学领域:
- 心脏病学 心脏病学
- 肺部病理学 肺部病理学
- 类风湿病学 类风湿病学
背景情况:
- 系统性硬化症相关的肺高血压 (SSc-PH) 的死亡率很高.
- 间歇性肺病 (ILD) 经常与SSc-PH共存,但其预后影响不同.
- 从ILD功能障碍在SSc-PH预后的作用是不清楚的.
研究的目的:
- 通过功能参数对SSc-PH患者进行分层.
- 在SSc-PH.内确定不同ILD模式中的预后因素.
主要方法:
- 对2015年以来诊断的SSc-PH患者的回顾性研究.
- 基于高分辨率计算机断层扫描 (HRCT) 和强迫生命能力 (FVC) (<70%或>=70%) 的分类.
- 使用Kaplan-Meier,后勤回归和ROC分析评估的生存分析,临床结果和预后因素.
主要成果:
- 对于SSc-PH的五年生存率为66.7%.
- PH-ILD患者的生存率比PAH患者 (87%) 低 (48%).
- FVC <70%显著降低了生存率 (29%vs. 80%) 并是唯一的死亡率预测因素.
结论:
- 在诊断时FVC<70%是SSc-PH的负预后因素,无论HRCT上的ILD程度如何.
- 功能性评估补充了SSc-PH风险分层的放射性评估.
- 常规的肺功能监测对于管理SSc-PH至关重要.
相关概念视频
Lung Capacity
56.2K
The air in the lungs is measured in volumes and capacities. Lung volume measures reflect the amount of air taken in, released, or left over after a lung function, like a single inhalation. Lung capacity measures are sums of two or more lung volume measures.
56.2K
Pulmonary Hypertension: Classification and Pathogenesis
593
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...
593
Overview of Systemic and Pulmonary Circulation
10.3K
The systemic and pulmonary circuits are crucial components of the circulatory system, working together to transport blood between the heart, lungs, and the rest of the body. The process begins with pulmonary circulation, where deoxygenated blood is pumped from the right ventricle to the lungs via the pulmonary trunk and arteries. Upon reaching the lungs, the blood becomes oxygenated and returns to the heart, specifically to the left atrium, via the pulmonary veins.
The oxygenated blood is sent...
The oxygenated blood is sent...
10.3K
Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors
578
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...
578
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
445
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...
445
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists
475
Prostacyclin receptor agonists are a class of therapeutic agents integral to managing pulmonary arterial hypertension (PAH). These drugs operate by mimicking the action of prostaglandin I2, or PGI2, a naturally occurring compound in the body.
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
475


