特发性肺间质纤维化(IPF)是一种病因不明、发病机制不清、缺乏治疗手段的致命性弥漫性肺间质疾病,其病理特征是肺泡上皮的损伤和成纤维细胞增殖,并形成成纤维细胞灶(fi-brob last foc i)[1],近年来国际上对特发性肺纤维化(IPF)的研究和认识进展很快,现就病理学特征、基础和临床 Idiopathic pulmonary fibrosis (IPF) is a fatal diffuse interstitial lung disease of unknown etiology, unclear pathogenesis and lack of treatment. Pathological features are the damage of alveolar epithelium and fibroblast proliferation. The formation of fibronuclear foci (fi-brob last foc i) [1], in recent years, international research and awareness of idiopathic pulmonary fibrosis (IPF) progressed rapidly, now on the pathological features, basic and clinical