Pulmonary Vascular Pruning on Computed Tomography and Interstitial Lung Disease Outcomes

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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.