In addition to patient-oriented research, we have 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 in order to identify key factors that drive the fibrotic process in ILD lungs.
Thromboxane-prostanoid receptor in pulmonary fibrosis. These studies are investigating the role of reactive oxygen species and signaling through the thromboxane-prostanoid receptor as a potential treatment for ILD.
Herpesvirus infection and immune dysregulation in pulmonary fibrosis. These studies are using an animal model of viral infection to investigate the role of adaptive immunity in lung 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.
AT2 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.
Joy Cogan, PhD
Jason Gokey, PhD
John Phillips, MD
Currently enrolling trials:
Celgene: CC-90001: Nationally, about 135 persons with IPF will enroll to receive either CC-90001 (at one of two doses) or placebo orally once daily for 24 weeks.
EMD Serono: Abituzumab: Patients with SScILD will enroll to receive either Abituzumab (at one of two doses) or placebo intravenously once every four weeks for 100 weeks.
TRAIL-1: pirfenidone: Patients with RA-ILD will enroll to receive either pirfenidone or placebo orally three times daily with meals for 52 weeks.
Biogen (SPIRIT): BG00011 vs. placebo: Patients with IPF will receive BG0001 or placebo by subcutaneous injection once weekly x 12 month.
Novartis: VAY736 vs. placebo: Patients with IPF will receive VAY736 or placebo by subcutaneous injection once montly x 12 month.
Pulmonary Trials Cooperative: CleanUP IPF: Patients with IPF will receive antibiotics (bactrim ds or doxycycline) daily x 2 years.
PULSE - iNO vs. placebo: Patients with IPF wearing oxygen >12 hours per day are randomized to wear a device administering placebo or inhaled nitric oxide x 16 weeks.