People affected by idiopathic pulmonary fibrosis (IPF) currently have limited treatment options to slow the progression of disease. New research studies are needed to gain valuable knowledge about potentially better treatment options.

People with IPF often have acid reflux, which is thought to result in stomach acid potentially reaching the lungs and causing damage. The production of stomach acid can be stopped by medicines called proton pump inhibitors (PPIs), which can then reduce the symptoms of acid reflux.

The aim of the TIPAL study is to see if IPF progresses at a slower rate when taking PPIs.

Many patients diagnosed with idiopathic pulmonary fibrosis experience a troublesome cough.  Treatment options are limited and clinical trials investigating better options are needed.

The IPF COMFORT study is investigating whether a study drug called orvepitant can reduce the burden  of cough in people diagnosed with IPF.

The cough in IPF may be caused by the airways becoming oversensitive. The investigational drug being tested in the study may reduce this over-sensitivity.

Malnutrition is when the someone doesn't get enough nutrients from food to meet the demands of the body. This can be due to a variety of reasons such as an increase in the energy requirements due to the lungs working harder. Or it could be due to the antifibrotic medication that can cause significant side effects, including poor appetite, diarrhoea, nausea, vomiting and weight loss.

The consequences of malnutrition can significantly impact quality of life, increase risk of infection and hospital admission, and is thought to potentially link with survival length. Very little research has been carried out to investigate how to best support people who are facing these challenges.

The DT-ILD study aims to find out if patients are willing to see a dietitian and if this can have a positive impact on their food intake.

Genetics can play a crucial role in the development and then progression of interstitial lung disease (ILD). This area of research is not fully understood and it is not known why some people who are exposed to pigeons, develop the disease, whilst others do not. ‍

The British Pigeons Fancier genetics of ILD study aims to find out more about the role that genetics plays in disease development of people with ILD who are regularly exposed to pigeons. This exposure is a known cause of hypersensitivity pneumonitis, which is a form of ILD.

Pigeon fanciers use their knowledge of pigeon genetics to breed winning pigeons. Researchers will use their knowledge of human genetics to investigate why some pigeon fanciers get pigeon lung. They will then compare genetic differences between pigeon fanciers with and without pigeon lung, to understand more about the disease.

Researchers would like to recruit people with regular racing pigeon exposure at national and regional pigeon fancier meetings.

The clinical trial is designed to see if a new drug called HZN-825, can help people with IPF. The aim is to understand how the body responds to the new drug, and if it affects lung function.

People affected by IPF currently have limited treatment options to slow the progression of disease. New research studies such as this, are needed to gain valuable knowledge about better treatment options.

The AIR Trial is investigating a new drug called compound 21 (C21) in people with IPF. This is a single treatment arm study, where all participants will take the drug.

Alveolar Epithelial Type II (AT2) cells, play a vital role in maintaining and repairing healthy lung tissue. People with IPF may have fewer of these AT2 cells, or the cells may not function correctly, which can promote scarring of the lungs. The new drug can attach to the surface of these cells and help to restore their normal function within the lungs.

The aim of the AIR Trial is to see if C21 can stabilise lung function in people with IPF and slow progression of the condition.

People affected by pulmonary fibrosis (PF) currently have limited treatment options to slow the progression of disease. New research studies are needed to gain valuable knowledge about potentially better treatment options.

The Fibroneer trial is a randomised controlled trial, designed to see if a medicine called BI 1015550 can help people with PF-ILD. The aim is to understand if the study drug affects scarring, inflammation and slows the rate of lung function decline.

PF-ILD is a group of progressive fibrotic interstitial lung diseases, that includes diagnoses such as: fibrotic hypersensitivity pneumonitis (fHP), rheumatoid arthritis interstitial lung disease (RA-ILD), and many others.‍

There is currently limited information and understanding about sexual function in patients with pulmonary fibrosis (PF).

‍This study aims to learn if sexual dysfunction is common in patients with PF.

The study will gain greater understanding about patients’ concerns and feeling around their sexual function. This knowledge will help to improve care and quality of life for patients.

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‍The aim of the study is to learn about how the body responds to a new drug Zelasudil (RX007) and if it is effective in slowing disease progression in people living with IPF‍

Measuring patients’ lung function is an important part of pulmonary fibrosis care. However, many factors can limit regular access to monitoring, including spirometry (breathing tests). The COVID-19 pandemic has presented additional challenges, highlighting a potential role for complimentary home monitoring options for patients. Home-based spirometry and digital technology mean that patients now have the opportunity to monitor their lung function at home.

The REMOTE-ILD study is investigating the impact of using a mobile app for home monitoring on patient care.

The study aims to understand whether monitoring lung function at home is helpful for patients and clinicians in managing pulmonary fibrosis.

In this study, the impact of regularly performing breathing tests at home in addition to usual (routine) care, is being compared to patients receiving their usual care alone. Participants will be randomly allocated to one of two groups, one monitoring their lung function at home in addition to their routine care, or and one to simply continue with their routine care. 

Progression of disease for people with fibrotic lung diseases, such as pulmonary fibrosis, is often unpredictable. Lung function test are part of standard care, and used to see how the disease is progressing over time. This doesn't always give a reliable picture of how things really are, as there can be difficulties when doing the tests.

High resolution computed tomography (HRCT) scans are used for diagnosis. Results are reported by trained radiologists, which can be limited by how each person interprets what they can see. A quantitative CT approach uses computer technology to analyse the HRCT results.

‍This study wants to understand if the new computer technology can predict and identify disease progression in fibrotic-ILD.‍

The study aims to see how the body responds to a drug Garadacimab and the effect it has on people with IPF. ‍

This study is finding out how a new drug, CHF10067, might affect people with IPF. The drug is a monoclonal antibody (MAB), which recognises and finds proteins on specific cells and help the immune system. Some patients who take part will be given the study drug and some will not.

The researchers want to learn how the new drug affects the body when it is given to people with IPF.

The study will learn about any side effects of the drug and understand how people respond to it.  

There are trillions of microbes, such as bacteria, fungi and viruses that live naturally on our bodies and inside us. This is known as our microbiome. It is thought that people with interstitial lung disease (ILD) have a microbiome that is different to those who are healthy.

This study aims to assess the microbiome of the gut and oral cavity in healthy individuals, so that any differences between people with ILD can be found. This may help researchers to identify potential screening tests that could allow for earlier diagnosis, or potential targets for treatments.

Pirfenidone is an antifibrotic medication that is often prescribed orally for people with IPF. The drug requires a large dose to make sure that enough reaches the lungs. This can cause significant side effects including abdominal pain, diarrhoea, sensitivity to sunlight, rashes, fatigue and liver problems. As a result of this, people with IPF often don't tolerate the drug or it has a large impact on quality of life.

Researchers have remade the drug so that it can be inhaled using a nebuliser. This means that small inhaled doses of pirfenidone can directly reach the lungs.

The ATLAS study aims to find out if inhaled pirfenidone is well tolerated and as effective as the oral medication.

The healthcare teams who look after people with pulmonary fibrosis should offer a personalised care plan to each person affected by the disease. However, it is not known which health aspects are the most important to people affected by pulmonary fibrosis.

A team of researchers have made a list of potential important health aspects. This list was made by gathering information from the research literature, healthcare professionals and patients. The list is currently too long to be of practical use within clinical care. Obtaining consensus on the most important health aspects (core outcome set) to use in clinical care is vital.

This study aims to shorten the list of the most important health aspects with the help of patients and caregivers. This will make it easier to use and implement within standard care.

Pulmonary rehabilitation is an exercise and education program that is recommended for people with certain chronic respiratory diseases, including pulmonary fibrosis.

Pulmonary rehabilitation classes often run over several weeks or months and can help patients to manage symptoms and cope with the effects of breathlessness on daily living activities. However, participation and completion of pulmonary rehabilitation often faces many different barriers.

The aim of this study is to design a financial incentive scheme to improve pulmonary rehabilitation referral, uptake and completion among people with chronic respiratory conditions and to design a study to investigate this scheme works.

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