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Pulmonary fibrosis means scarring (thickening) of the tissue of the lung.
Scarring is part of the body’s repair process and can help to heal injured areas. However, scar tissue that forms in the lungs can stop them working properly, making them less efficient at transporting oxygen into the blood and removing carbon dioxide. This often leads to breathing difficulties, which can be brought on by simple activities such as walking and talking.
Pulmonary fibrosis may be associated with a separate underlying disease, such as rheumatoid arthritis. When there is no known cause for its development, it is termed ‘idiopathic’.
These pages focus on idiopathic pulmonary fibrosis (IPF).
When you breathe in, air travels down your trachea (windpipe) and into smaller tubes that branch off your trachea, called bronchi (airways). These lead to your lungs, where they divide into thousands of smaller, thinner tubes called bronchioles that end in bunches of tiny air sacs called alveoli.
The alveoli act as the place for gas exchange in the body. Oxygen in the air you have inhaled (breathed in) passes through the thin walls of the alveoli into the tiny blood vessels (capillaries) surrounding the alveoli.
Once in the bloodstream, oxygen attaches to red blood cells and is transported to the rest of the body. In contrast, carbon dioxide is passed from the red blood cells into the alveoli and breathed out.
Sometimes, for reasons that are not entirely clear, the cells lining the alveoli can become damaged. These cells may try to heal themselves, but the healing process becomes uncontrolled, causing unwanted thickening and scarring (fibrosis) of the alveoli and the surrounding lung tissue. This affects the gas-exchange process and leads to a lack of oxygen in the body, producing breathlessness.
Cigarette smoking, certain lung infections and gastro-oesophageal reflux disease are just some of the possible factors that can cause damage to the cells lining the alveoli (for more information, see Pulmonary fibrosis – causes).
How common is idiopathic pulmonary fibrosis?
Idiopathic pulmonary fibrosis is rare, affecting fewer than 10 in 10,000 people in the UK. However, according to the British Lung Foundation, the number of cases seems to be rising. The reason for this is not clear.
The condition mostly affects middle-aged people aged 50 and over, and is more common in men and those who are smokers.
Idiopathic pulmonary fibrosis is a long-term condition and there is no known cure. Lung scarring in this condition is usually permanent.
There may be periods when the condition remains stable, but other times when symptoms (particularly breathlessness and cough) get worse.
Treatment aims to prevent further damage to the lungs. Oxygen therapy and pulmonary rehabilitation may help to improve the daily lives of some patients with pulmonary fibrosis (see Pulmonary fibrosis - treatment for more information).
Patients can also help themselves by leading as healthy a lifestyle as possible. This includes eating well, not smoking and exercising regularly up to a level they can tolerate.
In some cases, if the condition continues to get worse despite medical treatment, a lung transplant may be considered.
The most common symptom of idiopathic pulmonary fibrosis is breathlessness, which can get worse over time.
At first, many people ignore their breathlessness (especially older patients) and blame it on getting old or being out of shape. However, as the disease progresses and damage to the lungs becomes more severe, breathlessness can develop even with minor physical activity such as getting dressed or having a shower.
Less common symptoms of pulmonary fibrosis include:
In as many as half of cases, there is also a change in the shape of the finger and toenails. This thickening of the tissue at the base of the finger and toenails is known as clubbing. The exact cause of clubbing is unknown, but it can occur in people with various chest and heart diseases.
Heart failure can develop when the condition becomes severe. This is due to a combination of factors, including a build-up of blood in the right side of the heart (the side that pumps blood to the lungs), which develops because it is difficult to pump blood through the scarred lung tissue. The poor supply of oxygen from the diseased lungs to the heart muscle also contributes to heart failure.
Heart failure can cause various symptoms, including worsening breathlessness and fluid retention (oedema). For more information, see the Health A-Z page on Symptoms of heart failure.
‘Idiopathic’ means ‘having no known cause’. It is not known exactly how idiopathic pulmonary fibrosis occurs or why some people are more prone to developing it.
It is thought that the condition is triggered when the cells that line the lung alveoli (air sacs) become damaged. The affected cells try to repair themselves but the process becomes uncontrolled, resulting in thickening and scarring (fibrosis) of the alveoli and surrounding lung tissue. The amount of oxygen that can pass from the lungs into the blood is then significantly reduced.
Various factors have been suggested as possible triggers for the initial damage to the cells lining the alveoli, including:
There is currently no cure for idiopathic pulmonary fibrosis. The main aim of treatment is to improve the symptoms of the disease as much as possible and slow down its progression.
Medication can help to reduce symptoms in some people, but does not work for everyone. Some people, particularly elderly people whose symptoms are mild or not getting much worse, may be advised not to take any medication. This is because possible side effects could be worse than the progression of the disease.
Steroid medication is thought to improve symptoms in about one person in four with idiopathic pulmonary fibrosis. A low dose of a corticosteroid called prednisone may be prescribed. If there is no improvement after a few months, the treatment may be stopped.
Taking steroid medication, especially for a prolonged period of time, can cause side effects including:
It can also trigger diabetes and raised blood pressure. For more information, see the A-Z page on Side effects of corticosteroids.
Your doctor or specialist should always weigh up the potential benefits of steroid treatment against the risk of side effects.
Immunosuppressants are drugs that suppress the body’s immune system (thought to be partly responsible for idiopathic pulmonary fibrosis), helping to stop inflammation and fibrosis. You may be prescribed the immunosuppressant drugs cyclophosphamide or azathioprine.
Taking immunosuppressant drugs can increase your likelihood of getting other infections and can cause problems with your blood, such as anaemia.
Again, your doctor or specialist should always weigh up the potential benefits of immunosuppressant treatment against the risk of side effects.
A combination of steroids and an immunosuppressant may be used in some cases as there is thought to be more chance of improvement when these drugs are used together than when either medication is used on its own. Combination therapy also allows for the dose of steroids to be lower, meaning there is less chance of severe side effects.
When idiopathic pulmonary fibrosis is severe, the levels of oxygen in your blood fall and you feel more breathless. In this case your specialist may prescribe you oxygen, which can help with your breathing and enable you to be more active.
You will need to take oxygen through nasal tubes or a mask from a portable machine called an oxygen concentrator. This device provides air with a much higher oxygen level than the air you breathe in naturally. The tubes from the machine are long, so you will be able to move around your home while you are connected.
You can also use an oxygen inhaler for when you are out and about.
Pulmonary rehabilitation is a multidisciplinary treatment (involving many different types of healthcare professionals) used for many long-term lung conditions. Courses aim to help a patient come to terms with their condition, learn the best ways to deal with it and improve their ability to function on a daily basis without extreme breathlessness.
Courses in pulmonary rehabilitation are usually held locally and may include:
For more information, watch the above video in which a respiratory nurse specialist and patients explain pulmonary rehabilitation.
If your condition continues to get worse despite other forms of treatment, your consultant may recommend a lung transplant. The decision to have a transplant will be based on:
Few people with idiopathic pulmonary fibrosis are suitable candidates for a lung transplant.
As well as the medical treatments above, there are measures you can take to stay as healthy as possible:
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