摘要
该文主要介绍肺纤维化患者并发肺动脉高压(PAH)的发病机制、临床诊断方法以及目前对肺纤维化患者并发PAH的治疗手段。肺纤维化是一种慢性进展型肺疾病,主要表现为肺纤维组织过度形成和发展,患者在确诊后平均存活期<3年。PAH在血液动力学上定义为肺动脉平均压力>25mm Hg,且肺毛细血管压力<15mm Hg。肺纤维化患者因PAH并发症而加速纤维化病变,进一步破坏肺功能,缩短存活时间。近期研究表明,肺纤维化患者大多并发PAH,在纤维化早期发生率为33%,晚期则高达85%。虽然检测肺纤维化患者是否患有PAH仍然比较困难,但是通过X线胸透、超声心动图、心电图、肺功能检测(PFT)、运动(6min步行试验,6MW)以及血清脑钠尿肽水平可以初步监测PAH的发生,而且通过右心脏导管插入法(RHC)可以直接检测肺动脉压,进一步监测和诊断PAH。目前用于治疗肺纤维化引起PAH的药物包括磷酸二酯酶-5抑制剂、非选择性内皮素受体类似物以及前列环素类似物,但是治疗效果并不突出。所以,进一步研究肺纤维化引起PAH的发病机制,筛选潜在的靶向治疗位点,对患有PAH的肺纤维化患者的治疗十分必要。
In this review, we discuss the potential mechanisms of pulmonary fibrosis (PF) induced pulmonary arte- rial hypertension (PAH), summarize the prevalence of PAH in the setting of PF and current clinic methods for diag- nosis and treatment of these patients. PF is a chronic and progressive interstitial lung disease, characterized as for- mation or development of excess fibrous connective tissue in lungs. The average survival time of PF patients is less than 3 years after initial diagnosis. PAH is defined hemodynamically with a mean pulmonary artery pressure (MPAP) 〉25 mm Hg with a concomitant pulmonary capillary wedge pressure 〈15 mm Hg. The complication of PAH with PF will accelerate fibrogenesis, destroy lung function and decrease survival time. Recent reports revealed that PAH is in association with the processes of PF with an initial prevalence of 33%, and rose to 85% in latter stage. The diagnosis of PAH in the setting of PF remains difficult. Currently, the noninvasive modalities, such as chest imaging, cardiac echocardiography, pulmonary function test (PFT), exercise testing (6-minute walk, 6MW) and serum brain natriuretic peptide (BNP) levels may provide clues for diagnosis, and the invasive right heart cathe- terization (RHC) will provide direct evidence for final diagnosis. Current medications of PAH, such as phosphodi- esterase-5 inbibitors, nonselective endothelin receptor antagonists and prostacyclin analogues, have not shown satis- fying protection for PAH in the setting of PF. The treatment of PAH in the setting of PF is also difficult, and the further investigations of pathological mechanism and screening the potential therapeutic targets are essential for the treatment of PAH patients in the setting of PF.
出处
《中华高血压杂志》
CAS
CSCD
北大核心
2014年第9期804-807,共4页
Chinese Journal of Hypertension
关键词
肺纤维化
肺动脉高压
右心脏导管插入
肺动脉平均压力
Pulmonary fibrosis
Pulmonary arterial hypertension
Right heart catheterization
Mean pulmona- ry arterial pressure