Idiopathic pulmonary fibrosis (IPF) is a condition in which the lungs become scarred and breathing becomes increasingly difficult.
It's not clear what causes it, but it usually affects people who are around 70 to 75 years old, and is rare in people under 50.
Several treatments can help reduce the rate at which IPF gets worse, but there's currently no treatment that can stop or reverse the scarring of the lungs.
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The symptoms of IPF tend to develop gradually and get slowly worse over time.
Symptoms can include:
Many people ignore their breathlessness at first and blame it on getting old or being out of shape.
But eventually even light activity such as getting dressed can cause shortness of breath.
See a GP if you have struggled with your breathing for a while or have had a cough for more than 3 weeks.
These symptoms are not normal and should not be ignored.
If a GP thinks you could have a lung condition such as IPF, they can refer you to a hospital specialist for tests such as:
In people with IPF, the tiny air sacs in the lungs (alveoli) become damaged and increasingly scarred.
This causes the lungs to become stiff and means it's difficult for oxygen to get into the blood.
The reason this happens is not clear. Idiopathic means the cause is unknown.
IPF has been linked to:
But it's not known whether some of these factors directly cause IPF.
There's currently no cure for IPF, but there are several treatments that can help relieve the symptoms and slow down its progression.
IPF gets worse over time, although the speed at which this happens is highly variable.
Some people respond well to treatment and remain relatively free of symptoms for many years, while others may get rapidly worse or find the breathlessness debilitating.
It's very difficult to predict how long someone with IPF will survive at the time of diagnosis.
Regular monitoring over time can indicate whether it's getting worse quickly or slowly.
Before the availability of specific treatments like pirfenidone and nintedanib, about half of people with IPF lived at least 3 years from their diagnosis. Around 1 in 5 survived for more than 5 years.
It's hoped these figures will be improved by the availability of new treatments to slow the disease's progression.
If you have IPF, your clinical team will pass information about you on to the National Congenital Anomaly and Rare Disease Registration Service (NCARDRS).
The NCARDRS help scientists look for better ways to prevent and treat IPF. You can opt out of the register at any time.