Advances In Idiopathic Pulmonary Fibrosis-related Pulmonary Hypertension

Idiopathic pulmonary fibrosis (IPF) is a kind of unknownetiology, worsening of respiratory function, leading to a chronic respiratory failure fatallung disease. Its pathogenesis is not clear, large individual differences in diseaseprogression, lack of effective treatment, prognosis is poor, the median survival period isabout2.8~3.6years. Pulmonary hypertension (PH) is recognized as one of its seriouscomplications, its pathogenesis is still unclear and may include vascular damage,cytokines and growth factors cause excessive expression of hypoxic pulmonaryvasoconstriction and vascular remodeling. Research indicates that pulmonaryhypertension have significant negative effects impact on cardiac function and quality oflife in patients with IPF, increase mortality, reduce the average life expectancy, andsurvival period is less than1year. Currently, due to idiopathic pulmonaryfibrosis-related pulmonary hypertension associated clinical manifestations lack ofspecificity, and there is no validated standardized clinical approach to evaluate an IPFpatient who is suspected to have developed concurrent pulmonary hypertension. A lot ofIPF patients not clear diagnosis of pulmonary hypertension.Any hypoxemia andexercise capacity decline disproportionate with lung disease need further examination toevluate the presence of pulmonary hypertension.The treatment of pulmonary hypertension in patients with IPF is based on multiplefactors including disease severity, functional status and level of hypoxemia.This paperreviews based on the latest research of idiopathic pulmonary fibrosis-related pulmonaryhypertension.