特发性肺间质纤维化(Idiopathic Pulmonary Fibrosis,IPF)是一种病因不明、慢性进行性致死性疾病,发病机制尚未完全清楚,缺乏有效的治疗,预后较差,初次诊断后平均寿命约为3~5年。肺动脉高压(Pulmonary Hypertension,PH)是IPF的一种严重并发症,使IPF病情进一步恶化,肺活动耐量、一氧化碳弥散率及生活质量进行性下降,生存期缩短,平均生存期大多不 Idiopathic Pulmonary Fibrosis (IPF) is an unknown etiology and chronic progressive fatal disease. The pathogenesis is not yet fully understood, and there is a lack of effective treatment. The prognosis is poor. The average life expectancy after initial diagnosis is about 3 to 5 years. Pulmonary hypertension (Pulmonary Hypertension, PH) is a serious complication of IPF, which further aggravates the condition of IPF. The lung activity tolerance, carbon monoxide diffusivity and the quality of life decline progressively, the survival period is shortened, and the average survival time is mostly not