Idiopathic pulmonary fibrosis (IPF)

What causes IPF?

Idiopathic means the cause is not known but researchers now believe that the body creates fibrosis in response to injury to the lung.

The initial injury might be from:

  • acid reflux from the stomach
  • viruses. In some studies, IPF has been linked to certain viruses, including the Epstein Barr virus, which causes glandular fever. The herpes virus and hepatitis C have also been suggested as possible causes.
  • environmental factors such as breathing in certain kinds of dusts. It’s more common if you’ve been exposed at work to dust from wood, metal, textiles or stone, or from cattle or farming.

Some people may be genetically predisposed to develop IPF. But this link is only found in a minority of cases - about 10% in the UK in 2018.

Current drug treatments can slow the rate of scarring, but they do not stop it. Before the availability of specific treatments, studies showed that almost half of people with IPF in the UK died within 3 years of their diagnosis. But some people, about 1 in 5, lived for more than 5 years after they were diagnosed. Clinicians now believe the treatments available will mean that people diagnosed today will survive for longer.

Who gets IPF?

Latest research suggests about 6,000 people are diagnosed with IPF every year in the UK. Nearly 80% are men, and on average, about three quarters are over 73. 

Next: Symptoms of IPF >

 

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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.