特发性弥漫性肺间质纤维化是一种原因不明的广泛的间质性肺炎和肺间质纤维化,首先由Hamman和Rich于1935年报告,故曾称为Hamman-Rich综合征。以后的文献中使用的名称不一,如原因不明的纤维性肺泡炎、间质性肺炎-纤维化、特发性肺纤维化等。1959年,Beams等人建议将本病称为特发性弥漫性肺间质纤维化,此后大多数作者沿用这一名称。现根据近年来的材料综述如下。 Idiopathic diffuse pulmonary fibrosis is an unexplained, extensive interstitial pneumonitis and interstitial lung fibrosis that was first reported by Hamman and Rich in 1935 and was previously known as Hamman-Rich syndrome. Subsequent references used in the different names, such as unexplained fibrous alveolitis, interstitial pneumonia - fibrosis, idiopathic pulmonary fibrosis and so on. In 1959, Beams et al. Suggested that the disease be called idiopathic diffuse pulmonary fibrosis, and most authors followed that name. Now based on the material in recent years are summarized below.