Pulmonary Fibrosis

Pulmonary fibrosis is a lung disease that happens when lung tissue becomes damaged and scarred. It causes shortness of breath, a dry cough, and other symptoms.

What is pulmonary fibrosis?

Normal, healthy lungs are made up of soft tissue between lots of tiny air sacs (alveoli), which allow you to breathe in fully and deeply. 

In pulmonary fibrosis (PF), this tissue thickens and becomes scarred, meaning your lungs don’t expand as well as they should. It’s a permanent condition that usually gets worse over time, but certain treatments can help you manage your symptoms. 

Our lung specialists see patients with pulmonary fibrosis in our purpose-built Lung Health centre, where they offer the most advanced care in both diagnostics and treatment.

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The symptoms of pulmonary fibrosis often develop gradually, slowly getting worse over time. 

The signs to watch out for include:

  • shortness of breath

  • a persistent dry cough 

  • crackling in the chest

  • tiredness

  • aching muscles and joints

  • loss of appetite

  • unexplained or unintentional weight loss

  • clubbed fingers and toes

  • cyanosis (bluish, grey or white skin around the mouth or eyes)

Pulmonary fibrosis occurs when the thin walls of the alveoli (tiny air sacs in your lungs), and the areas around them, start to scar and thicken. This makes it harder for oxygen to pass into your bloodstream and be transported around your body.

Experts think this happens when your lungs don’t heal properly from damage or inflammation. You may be more at risk if you:

  • smoke

  • have acid reflux

  • have had a viral infection

  • have an autoimmune condition, such as rheumatoid arthritis, systemic sclerosis, or lupus

  • are often exposed to toxins, pollutants, and allergens in the air, such as silica, metal, asbestos fibres, bird feathers, and mould

  • have had chemotherapy or radiotherapy, or take medications such as antibiotics like nitrofurantoin or ethambutol

  • have a close relative with the condition – 1 in 20 people with pulmonary fibrosis has another family member with the condition

Sometimes a cause can’t be found, which is known as idiopathic pulmonary fibrosis (IPF). This is the most common type of pulmonary fibrosis that tends to affect men over the age of 55 who have smoked.

A diagnosis of pulmonary fibrosis is usually made based on your symptoms, medical history, and a physical exam, as well as after the results of certain tests, including:

  • lung function tests

  • blood tests

  • an X-ray or CT scan

  • bronchoscopy

  • a lung biopsy, but this rare

It’s not always possible to prevent pulmonary fibrosis, but you may be able to reduce your risk by:

  • avoiding substances that can harm your lungs – such as asbestos, metal dust, or chemicals. Wear a mask that filters particles from the air if you have to work with them

  • avoiding things that can cause chronic allergic reactions, like hay, grain, bird droppings or feathers, and heating and cooling systems

  • not smoking, or quitting smoking

If undiagnosed and unmanaged, pulmonary fibrosis can lead to many complications, such as:

  • high blood pressure in the lungs (pulmonary hypertension)

  • right-sided heart failure

  • respiratory failure – often the last stage of long-term lung disease, when blood oxygen levels fall dangerously low

  • lung cancer – pulmonary fibrosis increases your risk of developing lung cancer

  • other lung problems – such as blood clots in the lungs, a collapsed lung or lung infections

Pulmonary fibrosis is a disease that gets worse over time. While the condition can’t be cured, many treatments can help you manage symptoms and improve your quality of life. 

These include:

  • lifestyle changes – such as not smoking, eating a healthy diet, and exercising regularly

  • medications – specialist drugs like steroids can help, if your pulmonary fibrosis is caused by an autoimmune condition

  • oxygen therapy – using an oxygen mask can increase your oxygen levels, helping to make exercise easier, improve your sleep, and your wellbeing

  • pulmonary rehabilitation – physio-led exercises to help you manage and improve your breathing

  • a lung transplant – used in severe cases, but it’s rare

At Welbeck, our lung specialists are experts in their field and are dedicated to providing world-class care to every patient.

With access to colleagues across other specialties, our consultants are also able to refer within the Welbeck ecosystem if required to ensure you receive the treatment you need as quickly as possible, all under one roof.  

All appointments, testing, treatment, and follow-up appointments take place within our state-of-the-art facilities, enabling us to deliver accurate diagnostics and advanced treatments.


Your health is important to us, so we strive to offer same-day appointments whenever possible.

Our consultants are recognised by the major health insurance companies. If you have private health insurance, your treatment at Welbeck can begin once you have obtained authorisation. We also provide care to self-paying patients. Learn more about the different payment options at Welbeck.

Get in touch today to book an appointment.

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London

1 Welbeck Street
Marylebone
London
W1G 0AR

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Frequently asked questions

There’s no official staging system for pulmonary fibrosis, but the condition can be described as mild, moderate, severe, or very severe, depending on your symptoms, lung function tests, and imaging scans.

Lung function tests are used to find out how well your lungs are working. They most often include spirometry, where you breathe out quickly and forcefully through a tube connected to a machine. This machine then measures how much air your lungs can hold and how quickly air moves in and out of your lungs. A lung volume test is also included, which measures the amount of air your lungs can hold at different times, and lung diffusion tests, which show how well your body moves oxygen and carbon dioxide between your lungs and your blood.


Pneumonia is an infection that causes lung inflammation. It can lead to pulmonary fibrosis in some people, especially in severe cases, like those seen after certain viral or bacterial infections.


If you’ve been diagnosed with pulmonary fibrosis, it may be harder for you to fight off or recover from infections. Washing your hands often, disinfecting surfaces, and avoiding crowded places can help with this. Getting recommended vaccinations, like those for pneumonia, the flu, and COVID, can also reduce your risk.

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