What is idiopathic pulmonary fibrosis (IPF)?

Amanda Angus / 17 September 2019

More than 32,500 people in the UK are living with IPF and over 5,000 more are diagnosed each year.



IPF is an incurable, progressive and life-limiting lung disease in which the lungs become scarred and breathing becomes increasingly difficult. The scarring gets worse with time, and makes it hard to take a deep breath.

More than 32,500 people in the UK are living with IPF and over 5,000 more are diagnosed each year. The number of new cases is increasing – but it’s unclear what causes IPF (idiopathic means ‘of unknown cause’) and there is currently no cure.

The main symptoms of IPF (breathlessness and a cough) are often confused with those caused by other lung conditions, such as asthma or chronic obstructive pulmonary disease (COPD) or even heart problems. Therefore, it is often misdiagnosed. That means that life expectancy for people with IPF is lower than several common cancers.

Broadcaster and DJ, Janice Long, is keen on raising awareness of IPF, as this is the condition that her brother, the late television personality Keith Chegwin sadly passed away from in December 2017.

‘I had never heard of IPF in my life, I had no idea what it meant,’ Janice says. ‘I had to go online to find out the definition. It had never entered my world, my psyche, until Keith told me he had it. He said he had a lung disease – he called it ‘pulmonary fibrosis’. He said that he was one of the few people in the country that had got it. There was hope if he would get a lung transplant. He was obviously scared, there was no doubt about it, but he also had hope, which I think got him through.‘But there is no getting away from IPF. Once you have it, that’s it [unless you receive a lung transplant]. Life expectancy could be a couple of years, could be six years, but you can’t ignore it. Early diagnosis is obviously going to be beneficial and helpful to those around you – whether it be family of friends.

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‘Raising awareness is important because over 32,000 people are currently living with IPF, which is a lot more than Keith thought it was. More people die from IPF than die from stomach cancer or leukaemia. Raising awareness is key for making people think twice about any symptoms they might have, and for making people realise that it’s something that you have to pay attention to.

The main symptoms are shortness of breath and coughing with a dry cough. It’s best that you go and see your doctor to check it out. If you notice anybody who has these symptoms – send them to the doctor as soon as possible to get tested.’

Steve Jones, Chair of Action for Pulmonary Fibrosis, lived with IPF for eight years before receiving a lung transplant in 2016.

‘People should go to their GP if they have had a persistent cough for three weeks or more, or if they feel breathless doing household chores or walking up slopes or the stairs at home,’ he advises. 

‘About 35% of patients report being misdiagnosed with other conditions (eg asthma, heart failure) by their GPs. It is important that GPs listen to the patient’s chest thoroughly. IPF causes velcro-like crackles at the base of the lungs, which GPS can miss if they do not listen carefully.

‘Early diagnosis means that patients could be prescribed one of the two anti-fibrotic drugs, which have been shown to slow progress of the disease and can extend life by a year or more.

‘Early diagnosis may also mean that patients have the opportunity to go in a clinical trial for one of the pipeline drugs under development and they can be referred for pulmonary rehabilitation classes (exercise and education), which have been shown to be beneficial to their quality of life.

‘Epidemiologists estimate that the number of cases of IPF is increasing by 2-3% a year AFTER taking account of improved diagnosis methods and the fact that the population is raging - IPF is mainly a disease that affects people over 60.’

If you or someone you care about has been affected by IPF, the charity actionpulmonaryfibrosis.org is here to support you.



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