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Our IPF research

Idiopathic pulmonary fibrosis (IPF) is a lung disease that causes progressive scarring of the lungs. No one knows what causes it. That's where we come in. We want to know what causes it, because that will help us know how we can better treat it. We're funding research to do just that. 

We’re also funding research that looks to develop better treatment and care in IPF, and also develop a better understanding of the processes that lead to scarring. The results of this work could apply to the broader range of pulmonary fibrosis - conditions that involve lung scarring, including those where the root cause is known. We hope that this work will change the lives of people living with fibrotic lung diseases.

IPF in numbers

People blue outline
32,500

people live with IPF

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8,000

people are told they have IPF each year

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£ 2,316,086

total spend on all projects

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Aim of our IPF research

Although more and more people are being diagnosed with the condition, we still don’t know what causes it. We also have a limited understanding of how it leads to the lung scarring that is typical of this disease. Understanding the causes and biological processes that lead to this damage is key to developing better diagnostics, treatments and care for people living with IPF.

Our research into IPF is helping researchers to unravel the complex steps that are involved in the disease. And they’re pioneering the development of simpler diagnosis, better treatments and better care for people who have this condition.

IPF research projects

Here are some of the IPF research projects we've funded:

Changing the structure of the extracellular matrix to treat IPF

IPF occurs when lung cells in the lungs produce too much extracellular matrix (ECM), a kind of scaffold that supports cell growth and is also involved in the formation of scar tissue. Normally the ECM is produced and removed naturally by the body, but in IPF it builds up. Probably due to increased production, but also due to a failure to remove abnormal ECM. Too much ECM makes the lungs smaller, stiffer and less able to do their job of getting oxygen into the body.

During this study, Professor Simon Johnson and his team investigated ECM structure in people with IPF. They found that a specific enzyme that stabilises the structure of the ECM is overactive in IPF. They also found that blocking this enzyme increased the breakdown of ECM. This suggests that developing drugs that block this enzyme could reduce scar tissue in the lungs.

We hope that this study will lead to a clinical trial and the possibility of a new, safe and effective treatment for IPF. 

A lot of our work is trying to understand how the ECM in pulmonary fibrosis keeps the disease active. What we find is that the ECM produced from cells in patients with IPF causes the cells sitting on the ECM to grow more rapidly than normal. Once the disease starts, even more ECM gets deposited allowing more cells to grow faster. Professor Simon Johnson

A better way to treat and predict IPF

Research suggests that there are different drivers or mechanisms involved in IPF, which may help explain why it can affect people very differently in terms of how quickly it develops and progresses. It’s also suggested that in people with more aggressive IPF, the condition is already present and active in areas of the lung that appear to be normal. Since we have no way of detecting this, doctors find it difficult to predict the progression of IPF in each patient.

Professor Donna Davies

During this study, Professor Donna Davies and her team at the University of Southampton, looked at lung washings (a small amount of salt solution introduced into the lung, then suctioned out), blood and lung tissue samples from people with IPF. They investigated samples taken from both healthy and affected areas of the lungs to look for markers that could tell them more about the way that IPF develops in different people. The team found evidence of disease activity in areas of apparently normal lung tissue in people with IPF that is poorly detected with standard lung imaging. They also identified markers that may help to predict the course of the disease and have the potential to assist in early diagnosis of IPF.

More work is needed to investigate these findings further, but in the longer term, these markers may help doctors to identify people who are most likely to respond to a particular drug, helping them to give the right treatment to the right person. They will also provide researchers with a new measurement that will help them design more efficient clinical trials that will accelerate the development of new IPF therapies.

Because IPF is idiopathic - literally, we don’t know the cause - it is difficult to investigate the disease, because we have no starting point for it. So, we often work backwards. We look at somebody with IPF and try to understand features of the disease, in an attempt, eventually, to understand what causes it. Professor Donna Davies

Can reducing stomach acid improve symptoms in patients with lung scarring?

IPF is the term used for scarring of the lungs, when no cause for the scarring can be found. IPF leads to progressive breathlessness and cough. Dr Ian Forrest and his team at Newcastle University are looking into ways to reduce the cough that people with IPF have. Recent evidence suggests that small amounts of acid from the stomach can end up in the lung, producing tiny areas of scarring and the symptom of cough. Since stomach acid can be ‘switched off’ by using a drug called omeprazole, they’re doing initial tests in people with IPF to see if this drug reduces the persistent cough that some people with IPF have.

If the results are positive, this could lead to a trial on a much larger scale. It could be the world’s first proven, effective treatment for the debilitating cough that people living with IPF have.

Read more about Ian's research in his blog

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