How is pulmonary fibrosis diagnosed?

Pulmonary fibrosis is diagnosed through a number of different tests and is a joint effort from specialist teams. On this page we explain the tests you might have if it’s thought you might have pulmonary fibrosis.

What should I do if I have symptoms of pulmonary fibrosis?

Make an appointment with your GP if you have symptoms that might be due to pulmonary fibrosis.

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

If there is any suspicion you may have pulmonary 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 pulmonary fibrosis and ILD if there isn’t one in your local hospital.

What tests might I have to diagnose pulmonary fibrosis?

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. For example, 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

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.
  • 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’s only undertaken 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.
  • open lung biopsy is a procedure undertaken by a specialist surgeon under general anaesthetic to take a sample from the lung.

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 your test results to reach a diagnosis.

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