Interstitial Lung Abnormalities: Defining the Phenotype, Causes, and Consequences.

7.项目摘要/摘要 这项建议的主要目标是提供一个更准确的特征 预测肺纤维化(PF)发生和进展的因素 人口，目标是定义可能从早期参与中受益的群体 PF筛查研究。特发性肺纤维化(IPF)，最常见和最严重 肺纤维化(PF)的死亡率与许多终末期疾病相当。 尽管IPF历史上对 药物治疗，最近的研究表明，药物治疗可以减少 肺功能下降的速度，尤其是在早期开始的时候 疾病。我们最近的发现表明，早期发现肺泡炎是一种 可实现的目标。在PF患者的一级亲属中，我们已经展示了ILA 在我们评估的患者中，38%的患者和33%的ILA患者被发现有 更严重的疾病。这些亲属已被转介进行临床评估， 其中一些人已经开始对IPF/家族性PF进行抗纤维化治疗。而这些 研究结果表明，从反应到预防PF进展是一个里程碑式的转变 在近亲中是可能的，不知道在多大程度上可以检测到早期的PF 在其他处于危险中的独特人群中。基于这些发现，我们假设 可测量的特征可以在1)患有和不患有COPD的吸烟者中识别，2)在 早期肺癌患者，以及3)既往影像异常的患者， 这将有助于区分那些已经患有PF的人(和那些风险最高的人 从早期的PF进展)从那些不太可能发展成这种疾病的人。这个 这些研究的结果将提高我们对PF早期疾病检测的理解， 以及为招募这些特定人群进行试验做好准备 早期建立了新的和现有的医学疗法。

7. Project Summary/Abstract The primary objective of this proposal is to provide a more precise characterization of the factors that predict pulmonary fibrosis (PF) development and progression in specific populations with the goal of defining groups that might benefit from participation in early PF screening studies. Idiopathic pulmonary fibrosis (IPF), the most common and severe form of pulmonary fibrosis (PF) has a mortality rate comparable to that of many end- stage malignancies Although IPF has historically been unresponsive to pharmacotherapy, recent studies have demonstrated that medical therapy can reduce the rate of decline in lung function, particularly when started early in the course of disease. Our recent findings demonstrated that early disease detection for PF is an achievable goal. In 1st degree relatives of patients with PF we have demonstrated ILA in 38% of those we have evaluated, and 33% of those with ILA were found to have signs of more advanced disease. These relatives have been referred for clinical evaluations, some of whom have begun on anti-fibrotic therapy for IPF/familial PF. While these findings demonstrate that a landmark shift from reacting - to preventing – PF progression in close relatives is possible, it is not known the extent to which early PF can be detected in other unique populations at risk. Based on these findings we hypothesize that measurable characteristics can be identified in 1) smokers with and without COPD, 2) in patients with early stage lung cancer, and 3) in those with prior imaging abnormalities, that will help to distinguish those who already have PF (and those with the greatest risk to progress from early stages of PF) from those unlikely to develop this disease. The results of these studies will improve our understanding of early disease detection for PF, as well as setting the stage for trials aimed at the recruiting these specific populations for early institution of novel and existing medical therapies.