What is pulmonary fibrosis?

Pulmonary fibrosis is a condition where you have scarring (fibrosis) in your lungs. 

Pulmonary fibrosis is part of a larger group of lung diseases called interstitial lung diseases (ILDs). There are many different types of ILDs. Some of them can lead to pulmonary fibrosis, but not all of them do. 

Pulmonary fibrosis is a long-term condition. Any scarring that’s already in your lungs cannot be reversed. However, medicines and treatments can help slow down how much the scarring progresses, and keep your condition as stable as possible. 

How does pulmonary fibrosis affect the lungs?

It affects the tissue in and around the tiny air sacs in your lungs (interstitial tissue). These tissues provide structure and support. They also help oxygen and carbon dioxide move in and out of your lungs.

Pulmonary fibrosis makes it harder for the lungs to expand fully when you breathe in. It’s also harder for oxygen to move from the lungs into the blood. This means people with pulmonary fibrosis can have low blood oxygen levels.

DIAGRAM of lungs showing air sacs 

Progressive pulmonary fibrosis

Some types of pulmonary fibrosis can stay the same or change very slowly. But some types are progressive and get worse over time. 

• Idiopathic pulmonary fibrosis (IPF) is a progressive type of pulmonary fibrosis. Idiopathic means there is no known cause. IPF affects almost 40,000 people in the UK. It’s one of the most common fibrotic interstitial lung diseases (ILDs).
• Many other ILDs can also cause fibrosis and be progressive. These may be known as progressive fibrotic ILDs (PF-ILDs). They include hypersensitivity pneumonitis and sarcoidosis. 
• In some ILDs the fibrosis is a result of inflammation. Sometimes the fibrosis can be stopped with treatment and does not get worse. 

When you first get diagnosed, specialists may not be able to tell whether your condition is likely to be progressive or not, or how quickly it might change over time.

If your specialist team can work out what’s caused your condition, it will be easier for them to say if it’s likely to be progressive or not. 

They need to review your condition regularly and test your lung function. This helps them understand whether your pulmonary fibrosis is changing over time, and what this is likely to mean for you. 

How often you have reviews depends on the type of pulmonary fibrosis you have, how stable your condition is, your treatment, and your individual needs. 

Most people with pulmonary fibrosis have breathlessness. You may only notice this when you’re active, like climbing stairs. But some people feel breathless even when resting. Your specialist team can help you with breathlessness and any other symptoms you’re having.

Dr Matt Swallow, Asthma + Lung UK’s clinical lead.

The most common symptoms of pulmonary fibrosis are:

• feeling breathless with everyday activities or when resting
• a dry cough that does not go away and is not relieved by over-the-counter cough remedies
• feeling tired a lot, or all the time (fatigue)
• losing weight without trying to

See your GP as soon as possible if you notice any of these symptoms. 

Your GP may notice other signs, like finger clubbing, lips which are blueish or grey due to low blood oxygen levels, or crackling sounds when they listen to your chest with a stethoscope. 

Symptoms like breathlessness may be caused by other health conditions too. These include chronic obstructive pulmonary disease (COPD), heart disease, being overweight or becoming less fit as you get older. 

Pulmonary fibrosis can be caused by:

• breathing in dust from metal, stone and wood
• breathing in certain types of dust or organic matter, such as bird droppings
• drug side effects or treatment including some antibiotics, anti-inflammatory medicines and chemotherapy
• autoimmune conditions, like rheumatoid arthritis. 

You may have a higher risk of pulmonary fibrosis if: 

• you’re older - IPF mainly affects people over 50
• there’s a family history of ILDs, particularly IPF
• you smoke 

We’re finding out more about pulmonary fibrosis and its causes all the time. This means more people can find out why they developed it. But often the cause is not known. 

If you’re diagnosed with pulmonary fibrosis, it’s understandable to want to know what’s caused it, and upsetting when the answer is not as clear as you’d like. Whether you have idiopathic pulmonary fibrosis or a fibrotic interstitial lung disease, you need specialist care and management to help you live well with your condition. 

Dr Matt Swallow, GP and Asthma + Lung UK’s clinical lead.

The outlook for pulmonary fibrosis depends on what’s caused it, and whether it’s progressive or not. 

Pulmonary fibrosis is a feature of many different interstitial lung diseases (ILDs), and they can all have different outlooks. Some respond well to treatment and remain stable for many years.

Even progressive types of pulmonary fibrosis can have periods of time when not much changes and symptoms stay the same. 

And sometimes pulmonary fibrosis that’s been steady for a while can get worse suddenly. This is called an exacerbation or flare up.

Regular reviews help your healthcare professional see how your lungs are, how the scarring is progressing, and how it’s affecting your lung function. 

You can also get advice about lifestyle changes like quitting smoking, keeping active, and eating healthily, which can all help you live with pulmonary fibrosis and manage the condition.

Life expectancy for pulmonary fibrosis

Your specialist team can talk to you about how your condition is progressing, and how well you’re responding to treatments. 

Whatever type of pulmonary fibrosis you have, life expectancy depends on many different factors. 

These include:

• when and how quickly you were diagnosed
• whether you’re having any treatments like anti-fibrotics 
• how well you respond to treatments
• results of lung function tests
• your age
• your general health and fitness level, and if you smoke.

But it can be hard to accurately estimate life expectancy.

Life expectancy information online

You may have seen information online which says the average life expectancy of someone with pulmonary fibrosis is 3 to 5 years. 

This is from research into idiopathic pulmonary fibrosis which is quite old and out of date. Newer treatments are now working well to slow the progression of the conditionand improving outcomes for many people. 

This video made by the Pulmonary Fibrosis Foundation (U.S) has clear information about pulmonary fibrosis life expectancy. 

Some people tell us they want to know the stage of their pulmonary fibrosis so they can plan and understand how their condition is progressing. Others prefer to focus on living as well as they can day to day. 

Your specialist team can support you with whichever approach feels right for you and can make sure any conversations about life expectancy only give the amount of information you want.

Dr Matt Swallow, GP and Clinical Lead at Asthma + Lung UK.

You can call our helpline on 0300 222 5800.to talk to one of our respiratory nurse specialists 

Action for Pulmonary Fibrosis is a charity dedicated to supporting people with pulmonary fibrosis, their family and carers. It has a support line and a befriending service. You can also hear from other people living with pulmonary fibrosis.

The Pulmonary Fibrosis Trust supports anyone living with pulmonary fibrosis, with practical support, research and the chance to connect with others living with the condition.

Find out more about getting a pulmonary fibrosis diagnosis, treatments and how to manage the condition, and how to live well with pulmonary fibrosis.