Department of Radiologic Pathology and Pulmonary, Armed Forces Institute of Pathology, 6825 16th St NW, Bldg 54, Room M133B, Washington, DC 20306-6000, USA.
Radiology. 2010 Jun;255(3):692-706. doi: 10.1148/radiol.10090717.
The idiopathic interstitial pneumonias (IIPs) are a seemingly disconnected collection of diseases usually associated with the presence of pulmonary fibrosis. Categorization of the IIPs continues to be problematic despite recent attempts to refine the diagnostic criteria and suggests that rather than separate diseases, these pneumonias represent a spectrum of injury and abnormal repair of the alveolar wall. Although the initiating injury or injuries are unknown, the IIPs share a restricted number of final common abnormal pathways that lead to volume loss and lung distortion. The pathways include (a) alveolar collapse, (b) incorporation of fibroblastic material into alveolar walls, and (c) cigarette smoke-related inflammation and fibrosis. A collaborative diagnostic process in which data from radiologic and histologic assessments are combined allows a more reliable identification of the predominant pathways leading to pulmonary fibrosis. This approach has implications for therapy and the future direction of research.
特发性间质性肺炎（IIP）是一组看似互不相关的疾病，通常与肺纤维化的存在有关。尽管最近尝试细化诊断标准，但 IIP 的分类仍然存在问题，这表明这些肺炎不是单独的疾病，而是肺泡壁损伤和异常修复的一个连续谱。尽管起始损伤尚不清楚，但 IIP 有少数共同的终末异常途径，导致容积损失和肺变形。这些途径包括（a）肺泡塌陷，（b）成纤维细胞物质掺入肺泡壁，以及（c）与吸烟有关的炎症和纤维化。放射学和组织学评估数据相结合的协作诊断过程，可以更可靠地确定导致肺纤维化的主要途径。这种方法对治疗和未来的研究方向具有重要意义。
