肺动脉高压之结缔组织疾病

Connective tissue disease in pulmonary arterial hypertension

肺动脉高压(PAH)的特点是肺动脉压和肺血管阻力增加,可导致右心衰竭和死亡。几乎所有类型结缔组织疾病(CTD)都可并发PAH,称之为结缔组织疾病相关性肺动脉高压(CTD-PAH)。CTD是继发性PAH的第二大病因,国外最多见的是系统性硬化所致PAH,我国最常见为系统性红斑狼疮所致PAH。PAH可使CTD临床诊疗复杂化,并显著增加CTD患者死亡率。探索CTD-PAH发病机制、规范CTD-PAH诊疗有助于改善CTD-PAH患者预后。

Pulmonary arterial hypertension(PAH)is characterized by increased pulmonary arterial pressure and pulmonary vascular resistance,which may lead to right heart failure and death.Almost all types of connective tissue disease(CTD)can be complicated with PAH,which is called connective tissue disease-related pulmonary arterial hypertension(CTD-PAH).CTD is the second leading cause of secondary PAH.PAH caused by systemic sclerosis is the most common in foreign countries,while PAH caused by systemic lupus erythematosus is the most common in China.PAH can complicate the clinical diagnosis and treatment of CTD and significantly increase the mortality of CTD patients.Exploring the pathogenesis of CTD-PAH and standardizing the diagnosis and treatment of CTD-PAH are helpful to improve the prognosis of the patients with CTD-PAH.