Idiopathic pulmonary fibrosis, or IPF, is a devastating lung disease that causes rapid declines in lung function; up to 50% of patients will succumb to the disease within 3-5 years of diagnosis. Current treatment options have limited benefits, and do not reliably improve quality of life for patients.
Part of the problem is that the molecular and cellular mechanisms driving fibrosis in IPF have not been well studied.
Using a combination of imaging techniques, Dr. James Hogg’s group and their collaborators found a set of specific genes that are up- or down-regulated during fibrosis, compared to normal, healthy lungs. These specific changes are associated with the activation of immune responses and tissue repair processes.
These molecular and cellular changes likely trigger the development and pathology of IPF. These new findings could inform the development of better therapies for IPF.
Read the full study here, published in EBioMedicine.