Interstitial Lung Disease Research

Research Overview

The Interstitial Lung Diseases (ILDs) are a group of chronic, respiratory disorders characterized by persistent or progressive loss of functional alveoli in the lung and replacement with scar tissue.    

At Vanderbilt, we focus on understanding disease mechanisms that lead to Interstitial Lung Disease to improve ability to detect ILD at early stages and develop new treatment options for patients with ILD. For more than 20 years, we have been working to identify genes that confer susceptibility to pulmonary fibrosis, and more recently, we have been extending this work to investigate how environmental risk factors, together with inherited (genetic) risk factors lead patients to develop ILD. 

Our multidisciplinary research group integrates patient-centered clinical studies, state-of-the art genetic and genomic technologies, and innovative disease-modeling approaches in order to understand the cellular mechanisms that lead to ILD and identify novel approaches to intervene.  

Basic/Translational Research   

In addition to patient-oriented research, Vanderbilt has a very active basic science program utilizing patient-derived samples as well as innovative cell-culture and animal models focused on translating what we learn from patients into new approaches to treat or prevent ILD. 

Currently funded/active projects include:  

• Pulmonary fibrosis at single-cell resolution. These studies use lung tissue samples from patients with ILD and cutting-edge technologies that allow us to analyze the gene expression programs of individual cells to identify key factors that drive the fibrotic process in ILD lungs.  
• Primary cell cultures and organoid models with primary lung epithelial cells to model pathways that regulate lung injury and repair.  
• Studies involving immune dysregulation in pulmonary fibrosis.  
• Large-scale genetic studies involving familial pulmonary fibrosis.  
• Investigation of pre-clinical pulmonary fibrosis using a unique cohort of family members at-risk for development of familial pulmonary fibrosis 
• Molecular mechanisms of telomerase mutations and DNA-repair in IPF. These studies, using cell and animal models, are investigating how mutations in genes related to telomere biology and DNA-repair contribute to lung fibrosis.  
• Alveolar epithelial cell dysfunction in IPF. These studies, utilizing mouse models, cell culture, and organoid models are focused on understanding how cellular hypoxia and endoplasmic reticulum stress influence lung epithelial repair and remodeling.  

Faculty: 

• Timothy Blackwell, MD  
• Jason Gokey, PhD  
• Justin Hewlett, MD  
• Jonathan Kropski, MD  
• Lisa H Lancaster, MD  
• Margaret Salisbury, MD  
• Ana Serezani, PhD  

Clinical/Translational Research   

Vanderbilt has a very active ILD clinic caring for patients with IPF and other Interstitial Lung Diseases. Vanderbilt also recruits patients and their families into a number of ongoing clinical research studies supported by the NIH, Department of Defense, foundations, and industry partners. Through these studies, using blood or other samples from patients with ILD, researchers can gain new understanding into the causes and mechanisms of ILD.