特发性肺纤维化 (ｉｄｉｏｐａｔｈｉｃｐｕｌｍｏｎａｒｙｆｉｂｒｏｓｉｓ ,ＩＰＦ)是以弥漫性肺泡炎和肺泡结构紊乱最终导致肺纤维化为特征的间质性肺疾病 ,目前病因尚不清楚 ,主要表现是逐渐加重的呼吸困难 ,伴有刺激性干咳 ,病情一般持续进展 ,最终因呼吸衰竭而死亡。近年 Idiopathic pulmonary fibrosis (idiopathicpulmonary fibrosis, IPF) is an interstitial lung disease characterized by diffuse alveolitis and disorganization of the alveoli, which causes pulmonary fibrosis. At present, the etiology of the disease is not yet clear. The main manifestation is the worsening dyspnea, Accompanied by irritating dry cough, the condition is generally sustained progress, and eventually died of respiratory failure. in recent years