Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis

• About idiopathic pulmonary fibrosis
• Symptoms of idiopathic pulmonary fibrosis
• Causes of idiopathic pulmonary fibrosis
• Diagnosing idiopathic pulmonary fibrosis
• Treating idiopathic pulmonary fibrosis

About idiopathic pulmonary fibrosis

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 around 70-75 years of age 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.

Symptoms of idiopathic pulmonary fibrosis

The symptoms of IPF tend to develop gradually and get slowly worse over time.

Symptoms can include:

• shortness of breath
• a persistent dry cough
• tiredness
• loss of appetite and weight loss
• rounded and swollen fingertips (clubbed fingers)

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.

When to get medical advice

See your GP if you’ve struggled with your breathing for a while or have had a cough for more than three weeks.

These symptoms aren’t normal and shouldn’t be ignored.

If your GP thinks you could have a lung condition such as IPF, they can refer you to a hospital specialist for tests such as:

• breathing (lung function) tests
• blood tests
• a chest X-ray and computerised tomography (CT) scan
• a lung biopsy (where a small piece of lung tissue is removed during keyhole surgery so it can be analysed)

Read more about the tests for idiopathic pulmonary fibrosis.

Causes of idiopathic pulmonary fibrosis

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 isn’t clear. Idiopathic means the cause is unknown.

IPF has been linked to:

• exposure to certain types of dust, such as metal or wood dust
• viral infections
• a family history of IPF – around 1 in 20 people with IPF has another family member with the condition
• gastro-oesophageal reflux disease (GORD) 
• smoking

But it’s not known whether some of these factors directly cause IPF.

Treatments for idiopathic pulmonary fibrosis

There’s currently no cure for IPF, but there are several treatments that can help relieve the symptoms and slow down its progression.

Treatments include:

• self-care measures – such as stopping smoking, eating healthily and exercising regularly
• medication to reduce the rate at which scarring worsens – such as pirfenidone and nintedanib
• breathing oxygen through a mask – you can do this while you’re at home and/or while you’re out and about
• pulmonary rehabilitation – exercises and advice to help you breathe more easily
• a lung transplant – this is suitable in a few cases, although donor lungs are rare

Read more about how idiopathic pulmonary fibrosis is treated.

Outlook for idiopathic pulmonary fibrosis

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.

Other problems can also sometimes develop, including chest infections, pulmonary hypertension and heart failure.

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 three years from their diagnosis. Around 1 in 5 survived for more than five years.

It’s hoped these figures will be improved by the availability of new treatments to slow the disease’s progression.