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

间质性肺异常：定义表型、原因和后果。

基本信息

批准号：
10434099
负责人：
GARY MATTHEW HUNNINGHAKE
金额：
$ 82.07万
依托单位：
BRIGHAM AND WOMEN'S HOSPITAL
依托单位国家：
美国
项目类别：
财政年份：
2013
资助国家：
美国
起止时间：
2013-07-01 至 2025-04-30
项目状态：
未结题

来源：
https://reporter.nih.gov/project-details/10434099
关键词：
Acute Respiratory Distress Syndrome Address Adult Adverse event Affect Age Biological Markers Boston Cancer Patient Case-Control Studies Characteristics Chronic Obstructive Pulmonary Disease 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 Prognosis Pulmonary Fibrosis Radiology Specialty Reporting Research Respiratory Signs and Symptoms Risk Smoker Syndrome Work X-Ray Computed Tomography advanced disease adverse outcome antifibrotic treatment base chest computed tomography cohort design early screening experience fibrotic lung follow-up genetic predictors health care service utilization idiopathic pulmonary fibrosis improved insight interstitial lung volume mortality mortality risk novel prevent pulmonary function pulmonary function decline recruit research clinical testing respiratory screening stem transcriptomics

项目摘要

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/摘要该提案的主要目标是提供更精确的特征预测特定肺纤维化（PF）发生和进展的因素目标是确定可能受益于早期参与的群体 PF 筛查研究。特发性肺纤维化（IPF），最常见和最严重的疾病肺纤维化（PF）的死亡率与许多终末期患者的死亡率相当尽管IPF历来对阶段性恶性肿瘤没有反应药物治疗，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，早期发现 PF 疾病是一项可实现的目标。我们在 PF 患者的一级亲属中证明了 ILA 我们评估过的 38% 的 ILA 患者以及 33% 的 ILA 患者被发现有以下症状更晚期的疾病。这些亲属已被转介进行临床评估，其中一些人已开始针对 IPF/家族性 PF 进行抗纤维化治疗。虽然这些研究结果表明，从应对 PF 进展到预防 PF 进展发生了里程碑式的转变在近亲中是可能的，目前尚不清楚早期 PF 的检测程度其他处于危险中的独特人群。根据这些发现，我们假设可测量的特征可以在 1) 患有或不患有慢性阻塞性肺病 (COPD) 的吸烟者中识别，2) 早期肺癌患者，以及 3) 既往影像学异常的患者，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）到从 PF 早期阶段进展），避免那些不太可能患上这种疾病的人。这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进行试验奠定基础新的和现有的医学疗法的早期建立。