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What is Idiopathic Pulmonary Fibrosis (IPF)?

24 May 2016

Research has found that about 5,000 people are diagnosed with IPF every year in the UK.

Idiopathic Pulmonary Fibrosis (IPF) is one of many types of interstitial lung disease.

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‘Interstitial’ means that the disease affects the interstitium, a lace-like network of tissue that supports parts of your lungs.

The term ‘idiopathic’ means that the cause is not known.

When you have IPF, inflammation and scar tissue builds up in your lungs. This build-up of scar tissue is called fibrosis. As your lungs become stiffer and lose their elasticity, they are less able to take in oxygen from the air you breathe.

It is important to remember that no one is sure why people develop IPF, although it is more common in people who have been exposed to dust from wood, metal, textile or stone, or have been smokers. It is thought that in an unknown number of people diagnosed with IPF and have also been exposed to asbestos dust and fibres might in fact have an asbestos-related condition known as asbestosis.

Symptoms of IPF

The most common symptom of IPF is shortness of breath, especially when moving. It might occur when doing something that would not usually tire you out, such as walking upstairs or even just a short distance.

Because IPF usually occurs when people are older, you might think that you are just not as young as you used to be, but if you feel breathless, you should never ignore it and should see your doctor.

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Two other symptoms of IPF are the presence of a persistent cough and feeling tired all of the time.

Some people with IPF also suffer from:

  • fevers
  • weight loss
  • muscle and joint pain

“Clubbing” is another symptom that can be caused by IPF. You might not think that a lung condition could affect your fingers and toes, but this is often a distinctive sign of IPF. You might notice that:

  • Your nails feel too soft
  • Your nails feel as if they are coming loose
  • The shape of your nails change
  • The tips of your fingers or toes bulge out

Diagnosing IPF

If you begin to show symptoms of IPF, your GP should refer you to a consultant who specialises in lung conditions.

It is very important to identify the cause of your symptoms where possible, as this can greatly influence the choice of treatments.

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During diagnosis, your doctor should:

  • Listen to your chest
  • Ask questions about your medical history
  • Ask questions about your work history (this one is very important if you have ever been exposed to asbestos dust and fibres)
  • Order some blood tests to rule out possible known causes

Further tests that can be used to diagnose IPF include X-Rays and CT scans, biopsies to look inside the lung and breathing / lung function tests.

IPF and asbestosis

Asbestosis is the name given to a specific lung disease developed by people with a known history of exposure to asbestos. The symptoms and presentation of this disease can be identical to IPF.

Researchers have suggested that an unknown proportion of diagnoses of IPF may be due to exposure to asbestos. They analysed mortality rates for IPF, asbestosis and mesothelioma across England and Wales.

The analysis revealed national and regional correlations between the three diseases, which supports the theory that a proportion of IPF cases are due to exposure to asbestos but the person’s history of exposure to the deadly mineral has been missed or overlooked by medical professionals. If this asbestos exposure was known, it would be likely that these patients were diagnosed with asbestosis rather than IPF, and substantial financial help would potentially be available for them, which would significantly help with their care.

There were also high rates of IPF deaths in particular regions in the northwest and southeast of England, which has a history of shipyard work and therefore potential exposure to asbestos dust.

How do medical professionals diagnose asbestosis in cases of IPF?

As the first point of contact for most patients, medical professionals hold a pivotal role in the diagnosis of an asbestos-related disease, which could make all the difference to patients’ care and securing their families’ future.

To highlight asbestos-related diseases with medical professionals and as a gentle reminder for those GPs who are not aware of asbestos’ deadly legacy in the UK, we have put together six questions which may help with the diagnosis of an unknown lung condition:

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Hopefully, the six questions may save some time too.

Further reading

What is asbestosis?

What is asbestos?

Six questions that may establish whether an unexplained lung condition could be asbestos related


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