Symptoms of idiopathic pulmonary fibrosis 

The most common symptom of idiopathic pulmonary fibrosis (IPF) is shortness of breath, often when physically active, which can get worse over time.

At first, many people ignore their breathlessness (especially older patients) and blame it on getting old or being out of shape. But as the disease progresses and lung damage becomes more severe, even light activity like getting dressed or having a shower can cause breathlessness.

Other symptoms of pulmonary fibrosis include:

In up to half of all cases, there is also a change in the shape of the finger and toenails. This thickening of tissue at the base of the finger and toenails is known as digital clubbing. The exact cause of clubbing is unknown, but it can occur in people with various chest and heart diseases.

Secondary conditions

The strain IPF can place on your lungs and your heart can trigger a number of secondary conditions, outlined below.

Chest infection

You will have an increased risk of developing chest and airway infections such as:

Symptoms of a chest infection include:

  • worsening breathlessness
  • chest pain
  • a high temperature (fever) of 38C (100.4F) or above 
  • sweating and shivering

Pulmonary hypertension

Around a third of people with IPF will also go on to develop pulmonary hypertension. Pulmonary hypertension is when blood pressure inside the arteries connecting the lungs to the heart is dangerously high.

Symptoms of pulmonary hypertension include:

  • dizziness
  • feeling faint
  • chest pain (angina)
  • a racing heartbeat (tachycardia)

Read more about symptoms of pulmonary hypertension.

Heart failure

Heart failure, where the heart is unable to pump enough blood around the body, can develop when IPF becomes severe.

This is due to a combination of factors, including a build-up of blood in the right side of the heart (the side that pumps blood to the lungs), which develops because it is difficult to pump blood through the scarred lung tissue. The poor supply of oxygen from the diseased lungs to the heart muscle also contributes to heart failure.

Heart failure can cause various symptoms, including:

  • worsening breathlessness
  • extreme tiredness and weakness
  • swelling in the legs, ankles and feet (oedema) caused by a build-up of fluid

Read more about the symptoms of heart failure.


Page last reviewed: 04/07/2014

Next review due: 04/07/2016