Overview 

Idiopathic pulmonary fibrosis (IPF) is a progressive and irreversible condition in which the lungs become scarred, thereby leading to decreased lung function and breathing difficulties. With no cure, current treatment options for IPF primarily focus on alleviating symptoms, slowing the progression of the disease, and improving patients’ quality of life. In this blog post, we will cover everything you need to know about IPF - its causes, symptoms, and the current treatment landscape. We will also explore the pivotal role of clinical research in facilitating medical discoveries, and how your participation in an IPF trial, for which we are recruiting, could shape the future of IPF care.

What is IPF?  

In people with IPF, the tiny air sacs in the lungs (alveoli) are damaged, develop scar tissue, and cause the lungs to become increasingly stiff. This makes it difficult for them to expand and contract properly, a process vital for transferring oxygen from the air to the body, as well as removing waste gas from the blood. As such, IPF can limit the amount of oxygen in the blood and cause breathlessness from everyday activities like walking, getting dressed, and talking for extended periods. 

Causes

The term ‘idiopathic’ is used to describe any disease or condition for which the cause is unknown and, consequently, it is unclear why some people develop IPF while others do not. However, IPF has been associated with several risk factors, including: 

• Exposure to certain types of dust, such as metal or wood dust
• Viral infections
• A family history of IPF
• Acid reflux
• Smoking 

It is important to note, the exact relationship between these risk factors and IPF is still being investigated, and they may not directly cause the condition. 

Symptoms

As IPF is a progressive disease, its symptoms tend to develop gradually and get worse over time. These include: 

• Shortness of breath
• A persistent dry cough
• Tiredness
• Loss of appetite and weight loss

• Digital clubbing - Rounded and swollen fingertips caused by thickening of tissue at the base of the nail bed

Complications

IPF places enormous strain on the cardiovascular system, as the heart has to work harder to compensate for reduced oxygen levels in the blood. As such, IPF can increase a person’s risk of several health complications, including: 

• Bronchitis, pneumonia, and other chest infections, as scar tissue in the lungs can disrupt the respiratory system’s ability to effectively clear mucus and the contaminants it traps. 
• Pulmonary hypertension, a condition characterised by elevated blood pressure in the pulmonary arteries, which carry blood from the heart to the lungs. IPF can cause the pulmonary arteries to become inflamed and scarred, thereby increasing their resistance and forcing the heart to pump harder to push blood through the narrowed vessels. 

• Heart failure, when the heart is unable to pump blood effectively and results in the body’s organs and tissues receiving insufficient oxygen and nutrients. Over time, the strain imposed by IPF can change the heart’s structure and impair its pumping function.

Diagnosis

It is recommended to see your doctor if you have been experiencing prolonged breathing difficulties or have had a persistent cough lasting more than three weeks, as these symptoms are not typical and should not be overlooked.

If your doctor suspects IPF, they may refer you to a hospital specialist for tests to confirm the diagnosis, including: 

• Blood tests, as these can be used to determine the levels of oxygen and carbon dioxide in your bloodstream. 
• Breathing (lung function) tests, such as a spirometry test, wherein you breathe into a machine called a spirometer to measure how much air you can exhale in a single forced breath.
• A chest X-ray and CT (computerised tomography) scan, as these can help healthcare professionals confirm the presence of abnormalities, such as lung scarring, as well as exclude other potential causes of your symptoms, such as a build-up of fluid. 
• A lung biopsy - a medical procedure in which a small sample of lung tissue is extracted and examined under a microscope for signs of lung disease. 

Once a person receives a diagnosis of IPF, they will need to see their doctor and other healthcare professionals regularly to develop a personalised treatment plan and monitor their condition.  

Treatment

Currently, there is no mechanism to stop or reverse the scarring caused by IPF. As such, current treatment options focus predominantly on slowing the rate of scarring, relieving symptoms, and managing the risk of potential complications. The main treatment options include: 

• Medications, such as pirfenidone and nintedanib. These are anti-fibrotic and help slow the formation of fibrous tissue. 
• Oxygen support, where masks and other devices are used to administer supplemental oxygen to people whose natural breathing alone cannot provide sufficient oxygen. It helps alleviate breathing difficulties by improving oxygen levels in the blood. 
• Pulmonary rehabilitation, a structured program involving physical activity, breathing techniques, and education that can help people with IPF come to terms with and navigate their condition, as well as improve their quality of life. 
• Lung transplant, which may be recommended if other treatment options are no longer effective in managing symptoms or improving quality of life. 

Alongside medical care, there are several self-help techniques people can adopt to stay healthy while living with an IPF diagnosis, including: 

• Quitting smoking (if you smoke). 
• Keeping active (make sure to consult your doctor before starting a new exercise regimen, as they can provide advice about what types of exercise are safe and appropriate for your condition).
• Eating a healthy, balanced diet comprising a variety of nutrient-rich foods, such as fruits, vegetables, wholegrains, dairy, and lean proteins. 
• Staying up to date with your vaccinations for influenza and pneumococcal disease, as these conditions can be more serious if you have a pre-existing lung condition. 
• Whenever feasible, avoid close contact with people who have colds and/or other chest infections. 

How clinical research can help

In addition to current treatment options, your healthcare provider might recommend joining a clinical trial as a way to explore potential new treatments. Participating in a clinical trial offers a range of potential benefits, including: 

• Expert medical care and monitoring - Participants in clinical trials receive regular and thorough medical monitoring from healthcare professionals, thereby ensuring any changes or concerns are detected and managed promptly. 
• Contribution to medical advancements - By participating in a clinical trial, you could help researchers better understand the disease and improve treatment options for future patients.

 At time of writing, we are currently recruiting for a clinical trial investigating a potential new treatment for IPF. To find out more about this study, visit our website.