概述间质性肺疾病(ILD)是一组原因不同的弥漫性、非感染性、非恶性的下呼吸道炎症性疾病。ILD这一名词指的是肺泡间隙的增厚,通常为纤维化。事实上,ILD也还有其他的特征,如肺泡壁的上皮和内皮细胞排列紊乱,很多病例还有小气道和/或肺实质血管的紊乱。 ILD至少包括180种疾患,大约135种已知病因,45种病因不明。每一种均可结合临床、实验室、和形态学的特征而加以确定。这些疾病的自然 Summary Interstitial lung disease (ILD) is a group of diffuse, non-infectious, non-malignant lower respiratory inflammatory diseases of varying causes. The term ILD refers to the thickening of the alveolar space, usually fibrosis. In fact, there are other characteristics of ILD, such as alveolar wall epithelial and endothelial cells arranged disorder, many cases there are small airway and / or pulmonary parenchymal vascular disorders. ILD includes at least 180 conditions, about 135 known causes, and 45 etiologies unknown. Each can be identified in conjunction with clinical, laboratory, and morphological features. Nature of these diseases