Diagnosis and therapy of pulmonary hypertension in idiopathic pulmonary fibrosis

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. Key words: Idiopathic pulmonary fibrosis;  Pulmonary hypertension;  Diagnosis ;  Therapy