search Donate

What is pulmonary fibrosis?

The term pulmonary fibrosis (PF) describes scar tissue in the lungs.

This page explains how pulmonary fibrosis affects the lungs, why some people get pulmonary fibrosis, what the signs and symptoms are and what treatments are available.

Looking for this information in an alternative format? Discover our Navigating PF resources for anyone looking to learn more about pulmonary fibrosis.

What is Pulmonary Fibrosis?

Pulmonary - refers to the lungs

Fibrosis - refers to scarring

Pulmonary fibrosis is a condition where the lungs become scarred over time, making it difficult for the lungs to function properly. Symptoms often include shortness of breath, a dry cough, fatigue and weight loss. For many people, pulmonary fibrosis may get worse over time.

Pulmonary fibrosis is a type of interstitial lung disease (ILD). There are many different types of pulmonary fibrosis and ILDs. Not all pulmonary fibrosis will get worse and the rate of progression can vary significantly between different types and individuals. Your medical team may use different terminology to describe your pulmonary fibrosis. Please reach out to your healthcare team for clarification if you are unsure.

Some examples of different types of pulmonary fibrosis and ILDs include:

Non-progressive pulmonary fibrosis – the result of a one-off event. It does not get worse over time.
Progressive pulmonary fibrosis (PPF) – pulmonary fibrosis that gets worse over time.
Idiopathic pulmonary Fibrosis (IPF) – the cause of the pulmonary fibrosis is unknown. This is the most common type of pulmonary fibrosis.
Interstitial lung disease (ILD) – a group of over 200 diseases that affect the interstitium (including autoimmune ILDs, hypersensitivity pneumonitis and sarcoidosis). The interstitium is the space in the lungs between the air sacs (alveoli) and the blood vessels.

How does pulmonary fibrosis affect the lungs?

The lungs have two main functions:

Bring oxygen into the blood.
Remove carbon dioxide from the body.

Healthy lungs are spongy and stretchy so air can easily move in and out of them. When you breathe in, air enters your airways and travels down into the air sacs (alveoli) in your lungs. Oxygen and carbon dioxide move between the alveoli and the blood. The space between the alveoli and blood vessels is called the interstitium.

With pulmonary fibrosis, scar tissue forms in the interstitium. This scar tissue is thicker and makes the lungs stiff and less elastic. This can lead to shortness of breath, reduced lung capacity and if severe, impaired movement of oxygen between the air and the blood.

What causes pulmonary fibrosis?

Research into pulmonary fibrosis is still developing. From what research into pulmonary fibrosis has learnt so far, there are two processes that can lead to scar tissue forming in the lungs:

1. Post inflammatory scarring

Inflammation in the lungs happens when something causes irritation or injury to the lungs. This injury can leave a scar. For example, radiotherapy.

2. Lung cells aging too quickly

Pulmonary fibrosis can happen when cells in the lungs age too quickly. Accelerated ageing is caused by a combination of genes and environment. Genetics can affect how cells age, which is why pulmonary fibrosis sometimes runs in families. Prolonged inhalation of smoke or dust, for example from tobacco or occupational exposures earlier in life, can also cause accelerated ageing.

Risk factors

From our current understanding, there is no single cause of pulmonary fibrosis. Instead, we talk about different risk factors. Risk factors can lead to inflammation or lung cells aging too quickly.

Risk factors can include:

older age
family history (the genes inherited from your parents)
breathing in something that irritates the lungs, such as smoke or dust – this will usually happen repeatedly over a long time
some medications, for example Nitrofurantoin and some anticancer drugs
other conditions, such as autoimmune rheumatic diseases
Gastro-oesophageal reflux disease (GORD).

What are the signs and symptoms of pulmonary fibrosis?

The signs and symptoms of pulmonary fibrosis vary between individuals. They are also likely to change if the disease progresses.

Symptoms: Your experience of pulmonary fibrosis

Common symptoms can include:

shortness of breath (breathlessness)
dry cough
tiredness (fatigue)
unexplained weight loss

Signs: Abnormalities found on physical examination

A common sign is hearing crackles when listening to your chest with a stethoscope. This sounds similar to Velcro being pulled apart.

Another sign can be clubbing of fingers and toes (swollen and rounded finger and toe tips – see diagram opposite).

How is pulmonary fibrosis diagnosed?

The healthcare professionals involved in your diagnosis will gather information in different ways. This can include:

Asking questions about your symptoms and your personal and family medical history. This will be a very full and detailed history and will enable them to decide what tests might be needed.
Asking questions about situations where you might have breathed in something that can irritate the lungs at work.
A physical examination. This could include listening to your breathing with a stethoscope (both front and back of your chest) and a full body examination.
Further tests normally include pulmonary (lung) function tests or a high-resolution computerised tomography (HRCT) scan.
Blood tests, which may be required to be repeated periodically.

The results of these investigations, along with a discussion at a specialist ILD multi-disciplinary meeting (MDT), will help your healthcare team to make a diagnosis. Sometimes, additional procedures may be needed. These may include:

a bronchoscopy (a camera into the lungs)
or lung biopsy (taking a sample of lung tissue).

Your healthcare team will discuss this with you if they think it is necessary. There are several types of pulmonary fibrosis. Your medical team will use information from your diagnostic test results, alongside a detailed and full medical history, to decide which type of pulmonary fibrosis you have. Sometimes this only becomes clear by observing if and how the disease changes over time. In most cases, your medical team will check if your disease changes. This is to see if your pulmonary fibrosis is progressing. It’s important to know this to help decide on the right support and treatment for you.

How is pulmonary fibrosis treated?

Currently, we can’t stop pulmonary fibrosis. However, some treatments can slow down the rate of further scarring. Other treatments can help to manage your symptoms. Not all treatments are suitable for everyone, so your medical team will discuss the best treatment options for you.

Some people have pulmonary fibrosis that is not progressive and might not need treatment.

Possible treatments to reduce further scarring include:

Antifibrotic medication (nintedanib or pirfenidone).
Immunosuppressants and steroid medications are sometimes used to treat inflammation.
Lung transplantation.

Possible symptom management includes:

Pulmonary rehabilitation.
Techniques to manage breathlessness and cough (including medication and non-medication methods).
You may have a secondary condition that has the potential to increase symptoms, such as acid reflux, which may require medication management.
Oxygen therapy.
Palliative care, which helps you to manage your difficult symptoms enabling you to live well with a life-limiting condition.
Support to maintain a healthy lifestyle.

What does a healthy lifestyle mean?

Everyday choices can help you live well with pulmonary fibrosis. This might include:

stopping smoking
staying active
getting support for mental health
eating well
keeping up to date with flu, pneumococcal and COVID-19 vaccines if eligible. Please discuss this with your clinical team.

Read more about questions you can ask your healthcare team about living well with pulmonary fibrosis.

How does pulmonary fibrosis progress?

Pulmonary fibrosis can be a progressive condition. If you are
unsure if your pulmonary fibrosis is progressive, please discuss this with your clinical team, as not all pulmonary fibrosis progresses.

Your symptoms may stay the same for a long time, or you may deteriorate quickly. Your healthcare team will monitor your pulmonary fibrosis to see if it progresses. This may be done by repeating a series of tests including:

lung function tests
CT scans.

They will then adjust your treatment options accordingly. If you feel that your symptoms are worsening, it is important that you do not ignore this and that you contact your clinical team for an earlier review. Some potential signs that your pulmonary fibrosis is progressing include:

increased breathlessness
rapid weight loss
increased fatigue
any other significant changes to your ability to navigate your daily life.

Life expectancy

Reading about life expectancy can be difficult. You might want to read this information with someone you trust and who can support you. The APF support line is also here for you.

callCall our Support Line on 01223 785 725

emailEmail our Support Line team on supportline@actionfp.org

Read about pulmonary fibrosis life expectancy

Support from Action for Pulmonary Fibrosis

If you or someone you know is affected by pulmonary fibrosis, we're here to support you. We have a range of services that are free to access:

Join a support group

There are over 100 support groups across the UK offering emotional support, helpful information, practical guidance
and signposting for anyone affected by pulmonary fibrosis.

Call our Support Line

Our Support Line is available Monday to Friday, 9am to 5pm on 01223 785 725. Not ready to call? You can email supportline@actionpf.org instead.‍

Join our webinars on pulmonary fibrosis

Our Talking PF webinars include in depth talks, patient stories and insight from healthcare professionals and include topics such as managing fatigue and emotional wellbeing.

Explore our information resources

We provide a variety of information from telling people you have pulmonary fibrosis, to the benefits you might be able to access if you have pulmonary fibrosis. Our resources are available to download or order in print.

Resources for healthcare professionals

Action for Pulmonary Fibrosis is here to support healthcare professionals too. Explore the resources available on our Healthcare Professional Hub.

Stay in touch

We would love to stay in touch with you about our
work. Please sign up to be sent regular information,
including our e-newsletter and Insider magazine. For our latest news and updates, you can also follow us on our social channels. We're on Facebook, Instagram and LinkedIn.

Our website and resources provide general information only. We cannot provide medical advice, treatment or prescriptions, nor can we assess/decide what services or clinicians you should be referred to. Support with these matters will need to be provided by your existing healthcare professional teams.

APF is not responsible for any errors or omissions or for any loss or damage suffered by users resulting from the information published on our website or in our resources.

Tab ID:174