Idiopathic pulmonary fibrosis affects more than 32,000 people in the UK

£800k funding to investigate treatments for devastating lung disease

Researchers at the University of Exeter have won more than £800,000 in funding to study a deadly lung disease – idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis affects more than 32,000 people in the UK, and accounts for one per cent of all UK deaths - with patients having a life expectancy of only three to five years once diagnosed. The causes of the disease are not fully known, but it involves scar tissue developing abnormally in the lungs, which progressively reduces the patient’s ability to breathe.

The new funding from the Medical Research Council will allow researchers to investigate the age-related complications of the disease. They will seek to understand why some cells age prematurely, becoming what is known as “senescent”, which in turn triggers the excessive scar tissue formation in the lungs of pulmonary fibrosis patients. This research will also investigate whether the senescent cells could be targeted with precision delivery of an anti-inflammatory molecule called hydrogen sulfide, which previous research has indicated could be effective.

Dr Chris Scotton, lead researcher at the University of Exeter Medical School, said: “This is a really exciting project, developed with input from our incredible patient involvement group, and which may have a profound impact on the lives of patients living with pulmonary fibrosis. Tragically, the current treatment options for patients with pulmonary fibrosis are extremely limited. This research offers some hope that if we can better understand the underlying causes, we can investigate new treatment options and improve life expectancy for these patients.”

Hydrogen sulfide gas is naturally produced by cells in the human body, is anti-inflammatory, and can potentially act as a fuel source within cells. This suggests it could be of benefit to senescent cells - which trigger an over-exuberant inflammatory response ultimately leading to the development of excessive scar tissue.

The research will use a combination of cutting-edge laboratory techniques involving human cell models and samples from patients to understand how the disease develops as well as the impact hydrogen sulfide can have as a treatment.

Date: 17 November 2020