Pulmonary fibrosis is a progressive, life-long condition that causes damage to the lungs making breathing increasingly difficult over time.
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Pulmonary fibrosis affects around 70,000 people in the UK. The condition is a group of serious lung diseases that affect the respiratory system, gradually damaging the lungs over time. The disease causes scarring in the lungs resulting in them becoming hard and stiff, which makes it increasingly difficult to breathe.
In pulmonary fibrosis, the thin walls of alveoli (tiny air sacs in the lungs) and the areas around them start to scar and thicken. This affects the ability of the lungs to effectively take in and use oxygen. It’s not always clear exactly why this happens but risk factors are thought to include:
In cases where the cause of pulmonary fibrosis cannot be identified, the condition is referred to as idiopathic pulmonary fibrosis (IPF). This is the commonest type of pulmonary fibrosis. Patients who develop idiopathic pulmonary fibrosis are usually men who have smoked and are over the age of 55.
The symptoms of pulmonary fibrosis tend to develop gradually and slowly get worse over time. As a result, many patients are mis-diagnosed as having other lung conditions such as COPD which is smoking related lung disease. It is important to diagnose pulmonary fibrosis as early as possible so that the appropriate treatments can be considered.
Symptoms of pulmonary fibrosis include:
A specialist will make a diagnosis of pulmonary fibrosis based on your symptoms and a physical assessment, as well as the results of various tests including lung function tests, blood tests, X-ray or CT scans, bronchoscopy and rarely a lung biopsy.
Pulmonary fibrosis is a progressive disease (it gets worse over time) and the damage to the lungs is irreversible. While the condition cannot be cured, there are several treatments available that can help slow down the progression and improve your quality of life.
Treatments for pulmonary fibrosis include:
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