Wendy Preston: Look out for signs of idiopathic pulmonary fibrosis

More people die from IPF than are killed on the roads each year, but few nurses or doctors have much experience of the disease.

More people die from idiopathic pulmonary fibrosis than are killed on the roads each year, but few nurses or doctors have much experience of the disease.

An X-ray of the chest of an 82-year-old man with idiopathic pulmonary fibrosis.
 Picture: Science Photo Library

Idiopathic pulmonary fibrosis (IPF) is a disease that causes scarring in the lungs, limiting the amount of oxygen that gets into the blood.

Idiopathic means ‘unknown’ – we don’t know what causes IPF. Research has suggested it’s more likely to occur if you’ve been exposed at work to dust from wood, metal, textiles or stone, or from cattle or farming, or if you smoke.

IPF has been linked to certain viruses, including the Epstein Barr virus. The herpes virus and hepatitis C have also been suggested as possible causes. It generally affects people who are middle-aged or older, with 85% of patients over 70.


The most common symptom is breathlessness, a dry cough and a change in the shape of fingernails and toenails called clubbing. These symptoms can be debilitating, life-changing and limiting.

The number of people dying of IPF has increased six-fold in the past 40 years and the prognosis is worse than for many cancers. Only one in five people will live for more than five years after they are diagnosed.

It affects 32,500 people in the UK and kills about 5,300 a year, significantly more than the number of people killed on Britain’s roads.


There are two new drug treatments for IPF but there is still no cure, and access to holistic care that includes timely palliative care is essential. The British Lung Foundation has funded over a quarter of a million pounds of research into IPF.

IPF is one of the less common lung diseases and doctors and nurses will have seen few, if any, people who have it.

If someone has shortness of breath and it’s possible to hear ‘crackles’ in their chest through a stethoscope then this may be a sign of IPF. The crackles sound like Velcro being pulled apart and if heard a referral should be made to a chest specialist for further assessment by a multi-disciplinary team.

Wendy Preston is RCN head of nursing and a respiratory nurse specialist

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