特发性肺纤维化相关性肺高压的诊断与治疗

Diagnosis and therapy of pulmonary hypertension in idiopathic pulmonary fibrosis

肺高压（pulmonary hypertension，PH）作为特发性肺纤维化（idiopathicpulmonaryfibrosis，IPF）的严重并发症，预后极差。其发病机制目前尚未完全阐明，PH的发生、发展严重降低了IPF患者的生活质量并缩短了患者的生存期。但早期诊断及合理治疗可提高该病患者的生存率并改善患者的生存质量。近年，对IPF相关性PH的认识不断深入，现将研究的最新进展作一综述。

Pulmonary hypertension （PH） is recognized as a severe complication of idiopathic pulmonary fibrosis （IPF） with poor prognosis. The pathogenesis is incompletely understood. The development of PH has a negative impact on the quality of life of IPF patients and is associated with poor survival. However, the early diagnosis and effective therapy can improve the survival rate as well as the quality of life. Currently, the understanding of this disease is increasingly deepening. The recent studies of PH in IPF are reviewed as follows.