Gadofosveset-enhanced Lung MRI to Assess Disease Activity in Fibrotic ILD
This translational study will find out if certain imaging (picture) abnormalities are present in people with interstitial lung diseases (ILD). In particular, patients with idiopathic pulmonary fibrosis (IPF), non-specific interstitial pneumonia (NSIP), and connective tissue disease-associated interstitial lung disease (CTD-ILD) are of interest. IPF, NSIP, and CTD-ILD are diseases in which normal lung tissues is gradually replaced with fibrosis, or scar tissue, which causes difficulty breathing. The cause of these diseases is currently unknown and once fibrosis begins, it often worsens over time and does not respond well to the treatments that are currently available. The clinical course in people afflicted with ILD varies significantly where some people’s lung function remains stable for long periods of time, while others experience a rapid worsening of their disease. Experiments have shown that lung “vascular leak”, which is a process by which fluid and proteins leak from the blood vessels into the lungs, appears to play an important role in the development and progression of pulmonary fibrosis and that people with ILD have increased lung vascular leak. Furthermore, research suggests that the amount of this vascular leak may correlate with worsening of their disease. It is hoped that the findings from this study will support our belief that lung vascular leak is important for the development and progression of pulmonary fibrosis and that this lung MRI technique may be helpful in predicting which subjects with ILD are more likely to experience worsening of their disease.
This study will use the magnetic resonance imaging (MRI) technique of the lungs with the use of a contrast agent (dye) that binds to protein in the blood is believed to help detect the amount of vascular leak that is present in the lungs. This technique will allow us to explore the possibilities that lung vascular leak is increased in people with ILD and the possibility that the severity of lung vascular leak in people with ILD is associated with worsening of the disease.
The primary objective is to determine whether lung vascular leak, as determined by gadofosveset-enhanced lung MRI, is increased in the lungs of IPF, NSIP, and fibrotic CTD-ILD patients compared to healthy controls. The secondary objective is to determine whether the extent of lung vascular leak, as determined by gadofosveset-enhanced lung MRI, is increased in ILD patients with progressive disease compared to ILD patients with stable disease, as defined by changes in lung function tests and if the extent of lung vascular leak predicts disease progression. The tertiary objective is to assess ventilation and perfusion abnormalities in ILD patients compared to healthy controls, and correlate these findings to areas of fibrosis and vascular leak.