特发性肺纤维化合并肺动脉高压的发病机制和治疗进展

Progress of pathogenesis and treatment for idiopathic pulmonary fibrosis complicated with pulmonary hypertension

特发性肺纤维化的晚期常合并肺动脉高压,病理表现为肺血管重塑、增殖和炎症,肺动脉高压的出现加重原有肺部疾病症状,导致右心衰竭,死亡风险增加、预后差、社会及家庭负担重。特发性肺纤维化合并肺动脉高压目前病因尚不明确、治疗棘手。本文对特发性肺纤维化合并肺动脉高压的发病机制及治疗进展进行综述,以期为特发性肺纤维化合并肺动脉高压的研究和治疗方案的制定提供新的思路。

Pulmonary hypertension is a common complication of advanced idiopathic pulmonary fibrosis,with the pathological manifestations of pulmonary vascular remodeling,proliferation and inflammation.Pulmonary hypertension aggravates the symptoms of the lung disease,leading to right heart failure and increased risk of death,which is associated with poor prognosis and heavy social and family burden.At present,the etiology of idiopathic pulmonary fibrosis with pulmonary hypertension(IPF-PH)is unclear and there is lack of effective treatment.This article reviews the progress of pathogenesis and treatment in IPF-PH,in order to provide new insight into research and treatment for IPF-PH.