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Idiopathic Pulmonary Fibrosis (IPF)

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What is Idiopathic Pulmonary Fibrosis (IPF)?

Idiopathic pulmonary fibrosis, often shortened to IPF, is a scarring or thickening of the lungs, for which there is no known or identified cause.

Idiopathic = of unknown cause

Pulmonary = of the lungs

Fibrosis = thickening or scarring of the tissue

This scarring makes it more difficult for your lungs to expand and for oxygen to move into your blood vessels and carbon dioxide to move out. This leads to symptoms such as breathlessness and tiredness.

It is thought that certain triggers may start the process of damage and inflammation. These include:

  • Smoking
  • Some viral infections
  • Heartburn (acid reflux)
  • A family history of IPF

What are the symptoms of IPF?

Symptoms usually develop slowly over a number of years. The main symptoms are:

  • Feeling breathless
  • Dry, tickly cough
  • Tiredness
  • Weight loss
  • Enlargement of the tips of your fingers and toes; this is called 'clubbing'
Find out more about IPF and its signs and symptoms in the CHSS booklet Living with idiopathic pulmonary fibrosis (PDF).