Interstitial Lung Research
In addition to providing outstanding patient care, members of the Interstitial Lung Disease (ILD) Center also seek to advance the understanding of interstitial lung diseases, particularly those that result in pulmonary fibrosis (i.e. scarring), with the ultimate goal of helping to develop new, more effective treatments for these diseases.
We continue to be an active participant in clinical trials for idiopathic pulmonary fibrosis.
Lebrikizumab in Patients with Idiopathic Pulmonary Fibrosis
A phase II, randomized, double-blind, placebo-controlled study to assess the efficacy and safety of lebrikizumab in patients with idiopathic pulmonary fibrosis.
ClinicalTrials.gov identifier: NCT01872689
Site Principal Investigator: Barry Shea, MD
The Shea Laboratory
Dr. Barry Shea’s laboratory uses a translational approach to better understand the biological mechanisms which drive the development and progression of lung fibrosis, with a focus on the role of ongoing, repetitive lung injury in idiopathic pulmonary fibrosis (IPF). The Shea lab seeks to integrate information obtained from bench research studies using animal models and cell culture systems with the results of clinical investigations of human disease. Towards this end, Dr. Shea has established a longitudinal clinical database and a biorepository of blood, bronchoalveolar lavage (BAL) fluid, and DNA specimens from patients with IPF and other ILDs, and he collaborates closely with the Elias and Chun labs at The Alpert Medical School of Brown University.
The Elias and Chun Laboratories at Brown University
For more than a decade, the laboratories of Jack Elias, MD and Chun Geun Lee, MD have been working to define the mechanism of pulmonary fibrosis by generation and characterization of transgenic and null mutant mice and other genetically modified mice. As an animal model of pulmonary fibrosis, the Elias and Chun labs developed triple TGF-b1 transgenic mice that are expressed only in the lung in an inducible fashion. The studies using these mice identified that TGF-b induces extensive airway and alveolar remodeling with fibrosis and emphysema. A transcription factor Egr-1 is implicated in this TGF-b-apoptotic tissue response, which is critical to the subsequent generation of TGF-b-induced fibrotic and emphysematous responses. Genetic factors or modifiers and other mediators that control TGF-b-stimulated pulmonary fibrosis are now being actively investigated using a variety of animal models. The findings made in these laboratories are now extensively tested in the group of patients with pulmonary fibrosis and chronic obstructive pulmonary disease (COPD) in collaboration with physician scientists at Brown University.