Pulmonary Vascular Pruning on Computed Tomography and Interstitial Lung Disease Outcomes

项目摘要/摘要 间质性肺病(ILD)是呼吸道疾病的主要来源，作为一个群体， 全球领先的肺移植适应症。两种特别具有破坏性的ILD并发症是 进展性肺纤维化(进展性ILD)和ILD相关性肺动脉高压(ILD-PH)。虽然有效 这两种并发症现在都有治疗方法，迫切需要识别高危患者。 在并发症已经发生，治疗变得不那么有效之前。 肺血管疾病的一个已知特征是相对较小的血管丢失，称为 “修剪”和血管异常都与进行性ILD和ILD-PH有关。使用高级图像 分析计算机断层扫描(CT)，修剪可以使用现有的成像数据进行量化-因为 几乎所有的ILD患者在诊断时都会接受CT扫描，这可能会导致 ILD风险分层的广泛改善。 这个项目的主要焦点是确定CT上的修剪是否可以识别那些有进展性疾病风险的人 ILD和ILD-PH。这项研究将使用一组高度特征化的ILD患者的现有数据 来自一个专科诊所，还将招募一个新的预期队列来收集与ILD相关的后续数据 结果。这项建议将结合先进的定量图像分析工具、严格的流行病学 方法，以及一套与ILD相关的综合结果测量，包括肺功能测试、CT 成像、运动测试、调查分数、血流动力学评估和临床结果回顾，例如 住院和死亡。这项研究的结果将有助于确定CT修剪是否是一种非侵入性标记 高危ILD--这可能有助于ILD并发症的早期和更好的治疗，因此 预防发病率，促进ILD患者更好的长期健康。 在这个项目中，候选人将提高对定量血管成像的熟练程度 分析技术，学习关键的新方法技能，并获得设计和实践经验 实施前瞻性研究。候选人将由一个杰出的、多学科的团队进行指导 专家将提供严格的培训，辅之以正规的课程和个性化的职业生涯 发展支持。该项目在一个特殊和独特的机构环境中进行，并且 利用来自贝丝以色列女执事医疗中心、哈佛大学陈公共卫生学院、 布里格姆妇女医院应用胸部成像实验室和哈佛大学催化剂。数据、培训、 该奖项提供的职业发展机会将使候选人成为领导者和 独立调查员在应用胸部成像工具方面将提供信息并改善临床护理 患有慢性肺病的人。

PROJECT SUMMARY/ABSTRACT The interstitial lung diseases (ILD) are a major source of respiratory morbidity, and as a group are the leading indication for lung transplantation worldwide. Two particularly devastating ILD complications are progressive lung fibrosis (Progressive ILD) and ILD-related pulmonary hypertension (ILD-PH). While effective treatments are now available for both types of complications, there is a critical need to identify at-risk patients before complications have already occurred and treatments become less effective. A known feature of pulmonary vascular disease is relative loss of the smaller vessels, referred to as “pruning,” and vascular abnormalities are linked to both Progressive ILD and ILD-PH. Using advanced image analysis of computed tomography (CT) scans, pruning can be quantified using existing imaging data – since virtually all patients with ILD undergo CT scanning at the time of diagnosis, this could potentially lead to widespread improvements in ILD risk stratification. The key focus of this project is to determine if pruning on CT can identify those at risk for Progressive ILD and ILD-PH. This study will use both existing data from a cohort of highly characterized patients with ILD from a sub-specialty clinic, and will also recruit a new prospective cohort to collect follow-up data related to ILD outcomes. This proposal will combine advanced quantitative image analysis tools, rigorous epidemiologic methods, and a comprehensive set of ILD-related outcome measures, including lung function testing, CT imaging, exercise testing, survey scores, hemodynamic assessment, and review of clinical outcomes such as hospitalization and death. The results of this study will help determine if CT pruning is a non-invasive marker of high-risk ILD – which may potentially facilitate earlier and better treatment of ILD complications, thus preventing morbidity and contributing to better long-term health for people with ILD. During this project, the candidate will develop advanced proficiency with quantitative vascular image analysis techniques, learn critical new methodological skills, and obtain hands-on experience in the design and execution of prospective studies. The candidate will be mentored by an outstanding, multidisciplinary team of experts who will provide rigorous training, complemented by formal coursework, and personalized career development support. The project takes place within an exceptional and unique institutional environment, and leverages resources from the Beth Israel Deaconess Medical Center, Harvard Chan School of Public Health, Applied Chest Imaging Laboratory at Brigham and Women’s Hospital, and Harvard Catalyst. The data, training, and career development opportunities provided by this award will position the candidate to be a leader and independent investigator in the application of chest imaging tools that will inform and improve the clinical care of individuals with chronic lung disease.