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

间质性肺异常：定义表型、原因和后果。

• 批准号: 9890852
• 负责人: GARY MATTHEW HUNNINGHAKE
• 金额: $ 86.69万
• 依托单位: BRIGHAM AND WOMEN'S HOSPITAL
• 依托单位国家: 美国
• 财政年份: 2013
• 资助国家: 美国
• 起止时间: 2013-07-01 至 2023-04-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/9890852
• 关键词: Address; Adult; Adult Respiratory Distress Syndrome; Adverse event; Affect; Age; Biological Markers; Boston; Cancer Patient; Case-Control Studies; Characteristics; Chronic Obstructive Airway Disease; Cicatrix; Clinical; Detection; Development; Disease; Epidemiology; Exercise; Fibrosis; First Degree Relative; Framingham Heart Study; Funding; Gases; Genetic; Goals; Grant; Image; Impairment; Institution; Lung; Malignant Neoplasms; Malignant neoplasm of lung; Matched Group; Measurable; Measures; Medical; Natural History; Participant; Patients; Pattern; Pharmaceutical Preparations; Pharmacotherapy; Phenotype; Physiological; Population; Populations at Risk; Positioning Attribute; Predictive Factor; Prevalence; Pulmonary Fibrosis; Radiology Specialty; Reporting; Research; Respiratory Signs and Symptoms; Respiratory physiology; Risk; Smoker; Syndrome; Work; X-Ray Computed Tomography; advanced disease; adverse outcome; base; chest computed tomography; cohort; design; early screening; experience; follow-up; genetic predictors; health care service utilization; idiopathic pulmonary fibrosis; improved; insight; interstitial; lung volume; mortality; mortality risk; novel; outcome forecast; prevent; recruit; research clinical testing; respiratory; screening; stem; transcriptomics

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.

7.项目总结/摘要 本建议的主要目的是提供一个更准确的特性， 预测肺纤维化（PF）发展和进展的因素， 目标是确定可能受益于参与早期 PF筛选研究。特发性肺纤维化（IPF）是最常见和最严重的 肺纤维化（PF）的死亡率与许多终末期肺纤维化的死亡率相当， 尽管IPF在历史上对化疗无反应， 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，特别是在病程早期开始时， 疾病我们最近的研究结果表明，PF的早期疾病检测是一种有效的方法。 可实现的目标。在PF患者的一级亲属中， 在我们评估的患者中，38%的人和33%的ILA患者被发现有 更严重的疾病。这些亲属已被转诊进行临床评估， 其中一些人已经开始对IPF/家族性PF进行抗纤维化治疗。 研究结果表明，从反应到预防PF进展的里程碑式转变 在近亲中是可能的，尚不清楚早期PF的检测程度 在其他有风险的独特人群中。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，2） 患有早期肺癌的患者，和3）在先前具有成像异常的那些患者中， 这将有助于区分那些已经患有PF的人（以及那些风险最大的人）， 从PF的早期阶段进展）从那些不太可能发展这种疾病。的 这些研究的结果将提高我们对PF早期疾病检测的理解， 以及为旨在招募这些特定人群的试验奠定基础， 新的和现有的医学疗法的早期机构。