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The diagnosis of pulmonary fibrosis can be challenging. Sometimes people are misdiagnosed or wait a long time for the correct diagnosis.

A new technology called eNose may help to diagnose people living with PF. The technology involves looking at the compounds in the air breathed out by someone living with PF which has potentially useful information for diagnosis and understanding disease progression.

The ILDnose study is investigating if the new technology can be used to help with diagnosis and determine which type of pulmonary fibrosis someone has.

Although the COVID-19 vaccination program has proven very successful for most people, clinically vulnerable people, including those with pulmonary fibrosis, remain at a higher risk of COVID-19 despite having the vaccinations.  

Antibodies form an important part of the immune response which helps the body to fight infection. There is some evidence that this response to the vaccination is important for how effective the vaccination will be. It is not clear whether this is true in all clinically vulnerable groups and there appears to be differences between individuals with the same condition. There may be other factors that play a role, including other parts of the immune system, other health conditions, particular medicines, or things like age. ‍

This study aims to investigate which factor or combination of factors predicts an individual’s risk to COVID-19.

There are currently limited tests that can predict disease progression for people living with IPF and limited understanding of what the response to treatment will be like for each individual.

A molecule in the blood, called Krebs von den Lungen-6 (KL-6) is higher in people that have IPF, compared to those who do not have the disease. It is unclear, if blood levels of KL-6 change over time in people with IPF or in response to treatment with antifibrotics.

‍This study aims to monitor blood levels of KL-6 over 12 months in people who are newly diagnosed with IPF. This will help to understand whether KL-6 changes in relation to treatment with antifibrotics and/or progression of IPF.

Pirfenidone is an approved treatment for pulmonary fibrosis in tablet form. Higher doses are needed for the ingredients of a tablet to reach the lungs. These high doses can be associated with significant side effects.

Initial studies in patients with idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) suggest that inhaled pirfenidone may reduce side effects and still help to slow the progression of the disease.

This study aims to investigate if inhaled pirfenidone reduces side effects and helps to slow the progression of the disease for people with PPF.‍

Systemic sclerosis (SSc), also known as scleroderma, is an autoimmune disorder causing inflammation and scarring (fibrosis) that can affect multiple organs. Around 50% of people with SSc develop interstitial lung disease (SSc-ILD).

People with SSc have higher levels of molecule in the blood which promotes inflammation and fibrosis. This molecule is called tumor necrosis factor-like cytokine 1A (TL1A).  ATHENA-SSc-ILD is a Phase 2 trial of a new drug, PRA023 for people with SSc-ILD. PRA023 binds to TL1A molecules affecting how these molecules work.‍

The study aims to better understand the effect of the drug on the body and investigate whether it reduces inflammation and scarring of the lungs, slowing progression of SSc-ILD.

The European IPF Registry (eurIPFreg) is one of the largest databases in Europe for the long term collection of information about people with IPF. This also includes information collected from other patient groups with different lung diseases, in order to act as comparisons.

The aim of the register is to describe and understand what happens to people with IPF over time.

The aim of this study is to investigate the safety and effectiveness of a new drug Leramistat in slowing lung function decline in people living with IPF.

Some people with pulmonary fibrosis receive a lung transplant. With any transplanted organs, including the lungs, there is a risk of rejection. If this occurs and is not treated, the transplanted organ can become damaged and will stop functioning. It is difficult to know if lung transplant rejection is happening, and people who receive a transplant need frequent tests to detect this.

The SENTINEL study aims to find out if a small patch of skin transplanted at the same time as the lung, from the same donor, can help to detect signs of transplant rejection earlier. This is called a sentinel skin flap. The skin is transplanted to the lower arm and may act as a way to visually check for potential rejection, showing a red rash. Currently, for lung transplants the only way to determine rejection is by doing a biopsy of the lung. It may also help to protect the lung transplant itself from rejection damage.

Systemic Sclerosis (SSc), also known as scleroderma, is an autoimmune disorder causing inflammation and scarring (fibrosis) that can affect different organs. Around 50% of people with SSc develop Interstitial Lung Disease (ILD) or SSc-ILD.

This study will investigate whether a new drug belimumab can help reduce lung function decline and change to skin thickening and other symptoms that can impact quality of life.

People with pulmonary fibrosis often experience breathlessness and shortness of breath. These symptoms are often distressing and have an impact on dairy life. Current management options are limited.

This study aims to find out if breathlessness management training can help people with pulmonary fibrosis.

Interstitial lung disease (ILD) develops in over half of patients with systemic sclerosis (SSc). Whilst ILD remains stable in some people, at least a third have progressively increasing fibrosis. There is a pressing need for accurate indicators that identify people at higher risk of progression, needing immediate treatment to prevent further irreversible ILD; and also people at lower risk who do not need treatment.

This study will evaluate archived CT scans of 1,000 people with SSc-ILD; 500 from the Royal Brompton/Royal Free Hospitals and 500 from collaborating centres in Italy, Germany, and France. The researchers will assess survival and long-term lung function. They will then run analysis with computers.

One computer programme will work out the volume of the airways, which tends to be larger when there is fibrosis, and the researchers think that this may predict progression of ILD. Another computer programme will work out the volume of the blood vessels, which may predict the risk of developing pulmonary hypertension, which can be a major complication for people with SSc-ILD. A third computer programme will work out the overall amount of fibrosis, to assess whether this is a better predictor of survival and disease progression compared to traditional measures of lung function.

In the subgroup of people who have had repeat CTs over time, the researchers will also look to see if changes on CT scans can make prognostic estimates more precise. Together, these findings could provide the basis for doing a very large research study including many other centres across the world to ensure our results are applicable to all SSc-ILD patients.

The overall aim is to use novel imaging biomarkers to develop SSc-ILD specific computer programmes that predict what will happen for people with SSc-ILD.

Some people with fibrotic interstitial lung diseases (ILD) have normal oxygen levels at rest, but experience low oxygen levels at night whilst sleeping. Previous research has shown that people who experience this can have poorer disease outcomes. We do not know why this is or the nature of the full impact.

This research study will look at oxygen levels at different times of the day and night, and whilst exercising. It will help to improve our understanding of ILD, and may give us a better idea of why some people suffer with fibrotic ILD that gets worse over time.

The research team aims to measure oxygen levels at night when people are asleep and during 6-minute walk tests. Measuring oxygen levels when walking can help to better identify people with fibrotic ILD at risk of clinical deterioration compared to the current standard assessments within clinical practice.

The BTS Interstitial Lung Disease Registry is a national project where data for all fibrosing ILD, including IPF and sarcoidosis is collected into an online system. Patient registries are collections of healthcare data which are for people with specific diseases or conditions.

‍The aim is to provide an easy system for data collection from a large number of people affected by PF to learn about the disease.‍

The registry collects information about clinical characteristics, the burden of disease, impact on quality of life and the disease course specific to the UK population. The longer term aims are to provide quality information to clinicians and researchers to help improve guidelines and management of disease.

Scientists are investigating the causes of pulmonary fibrosis to help develop new treatment options. Higher levels of certain molecules in the lungs are related to the development of pulmonary fibrosis. Higher levels of a molecule called lysophosphatidic acid, (LPA), is an example. The idea is that injury to the lungs the production of this molecule leading to more scarring of the lungs. A new drug BMS-986278 may help to block this process and may slow down lung function decline.

In this next phase of research, scientists want to find out more about the safety and effectiveness of the drug in a larger number of people living with progressive pulmonary fibrosis and investigate the potential effects on lung function decline.

ASPIRE IPF is a Phase 2b trial looking at the impact of a new oral investigational drug, buloxibutid, for people with idiopathic pulmonary fibrosis (IPF). The study will look at how the drug impacts ability to breathe, along with other important measures like safety and quality of life.

Buloxibutid is thought to activate a natural repair system that may help to reduce scar formation (fibrosis) in the lungs of people with IPF.

The aim of this study is to understand more about the effects of buloxibutid on people with IPF and how it may impact their lung function.

Interstitial Lung Diseases often result in troublesome breathing symptoms, such as breathlessness and coughing. For some people, as the disease gets worsen, the oxygen levels in the blood becomes low. It is not yet known if people in the early stages of disease have low levels of oxygen in their blood during activity, or if this low oxygen level could trigger more scarring. Oxygen therapy can be a treatment option, but it is still unclear what difference this makes to symptoms or lung scarring. Immune cells that are found in the blood and lungs are an important line of defence against infections, but their role in scarring lung diseases and how they are affected by low oxygen levels is still unclear.

In this study, the researchers will look at defender (immune) cells to see how they work, whether they are affected by low oxygen and how they interact with other cells in the lungs that are involved in scarring.

The researchers will also explore the oxygen levels in the blood of people who have scarring lung disease and those who don't, as well as monitoring levels over a single day and night.

The study aims to:

1. Measure oxygen levels over a typical day and night for people with ILD and see how this compares to those without the condition.
2. Assess if oxygen levels over the day and night can help to predict the disease getting worse.
3. Look at how immune cells in the blood and lungs of people with ILD work, and whether low oxygen affects these cells.

Scientists are investigating the causes of pulmonary fibrosis to help develop new treatment options. Higher levels of certain molecules in the lungs are related to the development of pulmonary fibrosis. Higher levels of a molecule called lysophosphatidic acid, (LPA), is an example. The idea is that injury to the lungs the production of this molecule leading to more scarring of the lungs. A new drug BMS-986278 may help to block this process and may slow down lung function decline.

In this next phase of research, scientists want to find out more about the safety and effectiveness of the drug in a larger number of people living with idiopathic pulmonary fibrosis (IPF) and investigate the potential effects on lung function decline.

This study is a proof of clinical concept investigation to understand the effects of a new drug, BI 1819479, on the changes in lung function test results for people with idiopathic pulmonary fibrosis (IPF).

New treatments are needed that further reduce the decline in lung function, positively affect symptoms and improve quality of life for people with IPF. This trial will investigate the drug, BI 1819479, and to be used as stand-alone treatment, or in addition to standard care of taking antifibrotic medication.

This study aims to test whether BI 1819479 improves lung function in people with idiopathic pulmonary fibrosis (IPF).

Pulmonary fibrosis changes over time. Changes are often gradual, but sometimes someone affected by pulmonary fibrosis may experience a sudden change in their symptoms known as an exacerbation or acute exacerbation.

At diagnosis, it is not known if the changes will be gradual or faster than expected. There are currently no tests that can predict exacerbations, and the cause is often unclear.

‍The aim of the study is to ask people to report any deterioration in their symptoms, so these can be examined further with tests. This may help to identify specific changes in blood and sputum that can predict exacerbations or identify triggers.

Idiopathic Pulmonary Fibrosis (IPF) is a chronic lung disease in which scar tissue builds-up in the lungs and can cause significant difficulty with breathing. People with IPF can have high levels of an enzyme called tissue transglutaminase 2 (TG2) in their lungs. GSK3915393 is a new drug, which is being tested to see if it can help people with IPF. The drug is thought to work by blocking the action of TG2 enzymes in the lung. Researchers think that this may help to reduce further lung damage in IPF.

In this study, researchers aim to evaluate the safety and effectiveness of GSK3915393 in people with IPF. They will test to see if GSK3915393 can help slow down the reduction of lung health decline.

There is research available on wellbeing and social needs for people with chronic respiratory illnesses such as COPD. However, to date, research is lacking when looking at mental health and wellbeing factors for people living with idiopathic pulmonary fibrosis (IPF), particularly in Ireland.

This study aims to collect information in the form of an online survey. The survey consists of twelve questions, the majority of which are multiple-choice. The survey is designed to be completed in twenty minutes. Information that specifically identifies who someone is, will not be collected when taking part, and all data will be stored according to GDPR guidelines.

The aims to:

1. To understand more about the mental health and wellbeing of people living with IPF in Ireland.
2. To document the social support needs of people living with IPF in Ireland.
3. To explore factors that predict the mental health and wellbeing of people living with IPF in Ireland. This will include perceived social support, illness perceptions, and coping strategies.