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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.

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.

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.

Treatments remain limited for people with pulmonary sarcoidosis. Initial treatment often involves taking corticosteroids which can help to reduce inflammation in the body. Some people are also prescribed immunosuppressant drugs. These treatments are not always effective and can lead to unwanted side effects.

The RESOLVE-Lung study is looking at a new drug, called namilumab. This drug is a human monoclonal antibody (mAB) that researchers think could help to treat the underlying cause of the disease. The study aims to find out how the body responds to the drug and if it is effective for helping people with pulmonary sarcoidosis.

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.

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.

There is currently limited understanding about the experiences of air travel for people living with pulmonary fibrosis and the effect that is has. This study is an online survey about the flight experiences of people living with pulmonary fibrosis.

The study aims to learn how pulmonary fibrosis can impact air-travel, including pre-flight, during the flight and post-flight.

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.

A new drug, called Efzofitimod, is being studied in people affected by pulmonary sarcoidosis. The drug dampens immune responses with the aim of reducing inflammation and potentially preventing lung scarring from developing.

This study aims to investigate  in a larger group of people whether the drug Efzofitimod could help reduce the progression of pulmonary sarcoidosis and what the side effects are.

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.

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.

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.

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 molecules called integrins are found in the lung tissue of IPF patients. A new drug bexotegrast may help to block the role of integrins in the scarring process and slow disease progression.

In this next phase of research, scientists want to find out more about the safety and effectiveness of this drug in people living with idiopathic pulmonary fibrosis and investigate the potential effects on lung function decline, symptom improvement, and quality of life changes.

The CORAL study is understanding whether a drug called nalbuphine extended release (ER) could treat chronic cough in people with Idiopathic Pulmonary Fibrosis (IPF). The extended release means that the drug is slowly and consistently released over a period of time.

Cough is one of the most common symptoms for people with IPF and there are currently no approved treatments to help reduce it. Doctors sometimes prescribe medicines that may slow down the scarring process or medications to reduce the inflammation in the lungs. However, patients may not experience relief from coughing.

This drug has been tested in a small group of people.

‍The aim of this clinical trial is to confirm if similar results are found in a larger number of people with IPF. This will help researchers to understand how the drug works and whether different doses of the study drug, nalbuphine, may help to reduce cough for people with IPF.

There is evidence that shows sensor technology, can be used to help manage older adults’ lung conditions and reduce the number of emergency visits to the hospital. Examples of sensor technology can include finger pulse oximeters, smartwatches, Fitbits, and smart inhalers.

However, we currently don't know which sensor technologies, features and functions are preferred by older adults.

In this project, the researchers want to find out what features and functions of different sensor technology are preferred. This includes the design aspects of the sensor device, as well as how people would like to see their personal health data. This will be explored through an online survey.  

The aim of the online survey is to assess the needs and preferences of older adults for self-managing their chronic lung condition using sensor devices.

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.

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.

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.