Pulmonary fibrosis

How is pulmonary fibrosis diagnosed?

Go to see your GP if you have symptoms that might be due to pulmonary fibrosis.

If you have IPF, doctors listening to your chest can often hear crackles in your lungs that sound like opening velcro.

Your doctor will examine you, looking for other causes of breathlessness. They’ll listen for crackles in your lungs and arrange a chest X-ray if they hear any.

If there is any suspicion you may have lung fibrosis, your doctor will refer you to a chest specialist at your local hospital. You may be referred on again for further investigation, or specialist treatment, to a specialist in ILD if there isn’t one in your local hospital.

The hospital doctor will ask questions about your medical, family and work history and your symptoms. They will examine you, listen to your chest and assess the need for tests.

You may need tests such as:

For some types of pulmonary fibrosis, the results from a CT scan can be very clear and allow a diagnosis to be made.

On a CT scan, IPF often shows up as a distinctive pattern on the lungs. You might hear your doctor call this honeycomb lung. The image shows lots of empty pockets or bubbles appearing where more solid-looking lung tissue would normally appear.

Looking inside your lungs

To get more information to help make a specific diagnosis, your doctor may need to look inside your lungs and possibly remove some cells or tissue for testing. Different procedures can be used to get some lung tissue:

  • flexible bronchoscopy involves inserting a narrow tube through your nose or mouth, down into your lungs. You will have a local anaesthetic sprayed inside your nose and throat and often a sedative injection into your vein for your comfort. The tube has a camera on the end so the doctor can see inside your lungs.
    The doctor may flush some water through the tubing to remove and collect cells for analysis. Your doctor may also remove samples of tissue. These are called biopsies and are painless. This is usually an outpatient or day case procedure.
     
  • video—assisted thoracoscopy (VATS) involves surgery under a general anaesthetic to get a larger piece of lung tissue. A surgeon makes keyhole incisions in your chest for a video-assisted surgical telescope to enter, and to remove tissue samples from your lungs.
    It has higher risks than a bronchoscopy. It’s undertaken only if your specialist needs more tissue to make a diagnosis and treatment plan. Your doctor will discuss the risks with you. You’ll stay in hospital for a few days for this test.

Diagnosing pulmonary fibrosis is a joint effort by your specialist team. It will include doctors who are experts in lung conditions, surgery, X-rays and scans, and laboratory tests. The specialist nurse is an important part of this team and a good source of information and support for you. This multidisciplinary team (MDT) will review all of your test results to reach a diagnosis.

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What’s the outlook?

Most forms of pulmonary fibrosis tend to get worse over time. The likelihood of this happening and how quickly it happens varies with the type, but also varies from person to person. This makes it difficult to predict exactly how fast symptoms may progress and change. The aim of treatment is to slow down this rate of change. The chance of success depends on the exact diagnosis and how well you can tolerate treatment. You will be monitored and have regular tests of your lung function to look for changes.

In some people, pulmonary progresses very quickly, while others live with stable disease for many years. Some conditions progress at a steady rate. In others apparently stable conditions can change suddenly. This sudden worsening of symptoms is called an exacerbation.

There are no defined stages in pulmonary fibrosis, but doctors may talk about mild, moderate, severe or end-stage fibrosis.

Next: treatment for pulmonary fibrosis >

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Last medically reviewed: August 2019. Due for review: August 2022

This information uses the best available medical evidence and was produced with the support of people living with lung conditions. Find out how we produce our information. If you’d like to see our references get in touch.