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Pulmonary Fibrosis
- What is pulmonary fibrosis?
- Causes of pulmonary fibrosis
- How does pulmonary fibrosis affect the lungs?
- Symptoms of pulmonary fibrosis
- How is pulmonary fibrosis diagnosed?
- Treatment for pulmonary fibrosis
- Living with pulmonary fibrosis
A CHSS booklet on pulmonary fibrosis will be available in Autumn 2012. Until then if you want more detailed information please call our Advice Line on 0845 077 6000.
What is pulmonary fibrosis?
- Pulmonary is another way to describe the respiratory system
- Fibrosis is an overgrowth of scar tissue.
In pulmonary fibrosis there is inflammation and scarring of the lung tissue. Oxygen cannot go through scar tissue the same way as it can through healthy tissue therefore pulmonary fibrosis makes it hard for oxygen to enter the blood vessels.
Pulmonary fibrosis (sometimes called fibrosing alveolitis) is a chronic chest condition which means that it cannot be cured and usually worsens over time.
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Causes of pulmonary fibrosis
There are many different conditions that can cause, or are associated with, pulmonary fibrosis. These are grouped together under the terms 'diffuse parenchymal lung disease' or 'diffuse interstitial lung disease'. They causes are quite rare and include:
- Sarcoidosis: inflammation of tissue in one or many parts of the body, most often affecting the lungs, lymph nodes, skin and eyes
- Occupational exposure to substances such as asbestos, metal dust, mould spores and other allergens
- Drugs or toxic gases, e.g. nitrofurantoin, amiodarone, bulsulphan, bleomycin, methotrexate, and chlorine
- Radiation treatment for other conditions, e.g. breast cancer
- Connective–tissue diseases, e.g. rheumatoid arthritis, scleroderma, systemic lupus erythematosus, ankylosing spondylitis
- Genetic susceptibility
The main cause of pulmonary fibrosis is unknown. This is referred to as idiopathic pulmonary fibrosis (IDF), also known as cryptogenic fibrosing alveolitis (CFA).
There are several theories as to what may cause IDF or CFA, such as viral illness and allergic or environmental exposure including tobacco smoke. Another theory is that it is an autoimmune disease: this means that your own immune system is causing inflammation in your lung tissue. These potential causes are still being researched.
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How does pulmonary fibrosis affect the lungs?
Pulmonary fibrosis always starts with inflammation of the lung tissue. The inflammation may heal normally or may lead to permanent scarring of the lung tissue.
Scarring of the lung tissue prevents the normal transfer of oxygen (from the lungs into the blood vessels) to all the cells of the body. When the cells do not receive enough oxygen the lungs have to work harder and symptoms, such as shortness of breath and tiredness, may appear. Breathlessness will gradually get worse as the disease progresses.
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Symptoms of pulmonary fibrosis
Symptoms differ in severity from person to person and can include:
- Shortness of breath that may worsen on activity
- A dry cough, which can get worse with activity
- Aching muscles and joints
- Enlarged fingertips: this is called 'clubbing'
- Tiredness and / or weight loss
- Fever
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How is pulmonary fibrosis diagnosed?
If your GP is concerned about your symptoms you may be referred to a respiratory specialist who will examine you and ask questions about your occupation; your environment and travel history; your family, smoking and medication history and your past medical history.
You may need some tests including:
- A chest x–ray: this may show widespread shadowing in your lungs
- Breathing tests: to help rule out other causes of shortness of breath
- Lung scans or lung biopsy: to confirm a diagnosis or to assess the severity of your condition
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Treatment for pulmonary fibrosis
Pulmonary fibrosis is a chronic condition which means that it cannot be cured and usually worsens over time.
The main aim of treatment is to try to prevent further irreversible scarring from occurring. The treatment you receive will depend on what has caused your pulmonary fibrosis (if known) and what symptoms you have. Your doctor will talk to you about the benefits, and side effects, of any possible treatment.
- You may be given steroid drugs to try and reduce the inflammation in your lungs that is causing the scarring. Steroids may stop it from getting worse. This treatment works best at an early stage when there is a lot of inflammation and less scarring.
- Sometimes drugs called immunosuppressants are used. These drugs suppress your body's immune system and can help to reduce inflammation.
Sometimes a combination of both of these drugs is recommended.
- You may need oxygen therapy to help improve your breathlessness.
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Living with pulmonary fibrosis
- A structured programme of pulmonary rehabilitation can help to control your breathlessness and help make everyday activities easier. Breathing control advice and appropriate exercise may be offered.
- You should be immunised against flu and pneumococcal pneumonia.
- Chest infections should be treated promptly by your GP. Learn how to minimise the risk of developing a chest infection.
- If you smoke try to stop. Support for this is available with nicotine replacement therapies and local smoking cessation support groups. Ask your practice nurse or GP.
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