Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is an incurable lung disease that results in death within a few years of diagnosis. We’re developing new patient-specific treatments and methods of diagnosis for IPF. 

Key investigator: Prof Luca Richeldi

Key projects

  • The lung cancer drug Nintedanib has been approved by the US Food and Drug Administration, EU and UK authorities as a new IPF treatment, following our demonstration that it slows the progression of IPF, providing an alternative to the only existing licensed treatment for this condition.
  • Using a lung tissue model developed by Prof Donna Davies' group, we are also conducting a pre-clinical study of the cancer drug Romidepsin as a new IPF treatment.
  • IPF involves the accumulation of scarring (fibrosis) in the air sacs of the lungs, which acts as a barrier to oxygen/carbon dioxide gas exchange with the bloodstream. We are analysing the collagen fibres the integrin molecules thought to be involved in the formation of this scar tissue. As scar tissue is less resistant to radiation, we are also investigating the impact radiation has on IPF lung tissue.        
  • We are conducting a long-term study using an electronic recording of lung sounds to diagnose patients with the early stages of IPF.