Innate Immunity in IPF

This translational study will look to understand how certain cells in the lung contribute to the development of pulmonary fibrosis. The lung has immune cells and cells that line the airways that help your body fight bacteria and viruses. These cells can also cause inflammation (reaction of a part of the body to fight bacteria and viruses) and damage to the lungs that leads to scarring. The lungs also contain bacteria and viruses (called the microbiome) that are important to maintaining health. This microbiome can be altered in diseases like pulmonary fibrosis and affect how your lung immune cells and airway lining cells work. It is hoped that the information collected from this study will help obtain a better understanding of how lung cells work and may help to find new ways to treat people with pulmonary fibrosis.

This study consists of two visits, a screening visit and a bronchoscopy visit, looking at patients with mild to moderate idiopathic pulmonary fibrosis (IPF) and healthy subjects with no history of lung disease. The screening visit involves assessments such as clinical safety labs, pulmonary function testing (PFT), electrocardiogram (EKG), and medical record review to ensure subjects fall within the inclusion and exclusion criteria of the study. The bronchoscopy visit involves a bronchoscopy procedure where cells from the lungs will be collected through bronchial alveolar lavage (BAL) and endobronchial brushing. In addition, specimens will be collected during this visit. These cells will be studied to help better understand how different types of cells contribute to the development of pulmonary fibrosis.

The primary objective is to characterize the immune cells and genetic profile in the lung. The secondary objective is to characterize the role of these immune cells on the induction of injury and fibrosis in the bronchial epithelium. The tertiary objective is to determine whether changes in the lung microbiome correlate to the immune cell and/or epithelial cell dysfunction.

Participating Clinics at Member ILD Centers