Idiopathic pulmonary fibrosis statistics

Idiopathic pulmonary fibrosis (IPF) is one of many types of interstitial lung disease (ILD). Interstitial means that the disease affects the interstitium, a lace-like network of tissue that supports the air sacs (alveoli) in your lungs. Idiopathic means the cause of the condition is not known. IPF is the most common type of ILD. Another, older, name for IPF is cryptogenic fibrosing alveolitis.

When you have IPF, scar tissue builds up in your lungs, making them thick and hard. This is called fibrosis. Fibrosis makes it harder for your lungs to take oxygen from the air you breathe. 

The causes of IPF are not understood in detail. But it is more common if you have been exposed through your occupation to dust from wood, metal, textile or stone, or from cattle or farming. Infection with particular viruses might be another cause.

There is no cure yet for IPF, although new drug treatments have recently become available.

These statistics on pulmonary fibrosis in the UK were compiled as part of our Respiratory Health of the Nation project by teams at St George’s, University of London, Nottingham University and Imperial College London.

Note on IPF prevalence and incidence data

IPF prevalence and incidence data need to be treated with caution. This is due to:

  • The range of possible codings that could be used by health care professionals to record IPF.
  • The changes that have been made to the way IPF is coded. These take time to become standard practice and therefore impact on the data recorded.

To decide which codings to use, we consulted leading epidemiologists and IPF clinicians. Other interpretations of the base data may yield different results.

The primary care codes that we used to identify IPF were:

  • H563.00 : Idiopathic fibrosing alveolitis
  • H563.11 : Hamman-Rich syndrome
  • H563.12 : Cryptogenic fibrosing alveolitis
  • H563.13 : Idiopathic pulmonary fibrosis
  • H563100 : Diffuse pulmonary fibrosis
  • H563300 : Usual interstitial pneumonitis
  • H563z00 : Idiopathic fibrosing alveolitis not otherwise specified
  • H563200 : Pulmonary fibrosis

Incidence figures for 2012 have been omitted from some of the analyses. This is because changes in the diagnostic coding used to record the disease led to a significant rise in recorded incidence.

It is unlikely that this reflects a real change in incidence. With the above caveats, our clinical and epidemiological experts believe the figures presented here provide an accurate picture of IPF in the UK.

Numbers of people with IPF

How many people in the UK have IPF?

In 2012, about 32,500 people had IPF in the UK.

In 2012, around 50 people in every 100,000 had been diagnosed at some time in their life with IPF, up from 37 people for every 100,000 in 2004.

Estimated numbers of people with idiopathic pulmonary fibrosis 2004–12

Number of living people per 100,000 ever diagnosed with idiopathic pulmonary fibrosis, 2004-2012

How many people have IPF in each region of the UK?

Prevalence is generally highest in Northern Ireland, north-west England, Scotland and Wales. IPF is least common in London. The reasons for this are not known. Yorkshire and Humber has one of the highest incidence rates of IPF in 2012. This is not in line with previous years, and could be related to how the data were recorded, rather than reflecting true IPF rates.

Number of people per 100,000 ever diagnosed with idiopathic pulmonary fibrosis, by UK region, 2004–12

How many people are diagnosed with IPF each year in the UK?

A small number of people are newly diagnosed with IPF each year. In 2012, 12 people for every 100,000 received their first IPF diagnosis, compared with 9 for every 100,000 in 2004.

Note: Incidence figures for 2012 are higher than for previous years. This is because changes in the diagnostic coding used to record the disease led to a significant rise in recorded incidence. It is unlikely that this reflects a real change in incidence in this year

Number of people per 100,000 newly diagnosed with idiopathic pulmonary fibrosis, each year, 2004–12

How many people were diagnosed with IPF in each UK region in 2004–12?

Due to the relatively small numbers of people newly diagnosed with IPF, regional differences fluctuate by chance from year to year.

Note: Incidence figures for 2012 are higher than for previous years. This is because changes in the diagnostic coding used to record the disease led to a significant rise in recorded incidence. It is unlikely that this reflects a real change in incidence in this year.

Number of people per 100,000 newly diagnosed with idiopathic pulmonary fibrosis, by UK region, 2004-12

How many males and females have IPF in the UK?

In 2004-12, more males than females had IPF. In 2012, 62 males and 40 females for every 100,000 had been diagnosed with IPF at some time in their life.

Number of males and females ever diagnosed with idiopathic pulmonary fibrosis per 100,000, 2004–12

How many males and females were diagnosed with IPF each year during 2004–12?

Throughout 2004-12, more males than females were newly diagnosed with IPF each year. In 2012, 16 males and 9 females for every 100,000 were first diagnosed with IPF

Note: Incidence figures for 2012 are higher than for previous years. This is because changes in the diagnostic coding used to record the disease led to a significant rise in recorded incidence. It is unlikely that this reflects a real change in incidence in this year.

Number of males and females per 100,000 newly diagnosed with idiopathic pulmonary fibrosis each year, 2004–12

Ages of people with IPF

How old are the people with IPF in the UK?

The people who get IPF are mostly over the age of 40, and the chances of diagnosis increase significantly as people get older.

Number of people per 100,000 ever diagnosed with idiopathic pulmonary fibrosis, by age group, 2004–12

How old are the people who are diagnosed with IPF each year in the UK?

Almost all of the people who develop IPF each year are over 40. In 2004-12, the chances of developing the condition increased as people got older.

Note: Incidence figures for 2012 are higher than for previous years. This is because changes in the diagnostic coding used to record the disease led to a significant rise in recorded incidence. It is unlikely that this reflects a real change in incidence in this year.

Number of people per 100,000 newly diagnosed with idiopathic pulmonary fibrosis, by age group, each year, 2004–12

Deaths from IPF

How many people died from IPF in the UK in 2012?

In 2012, 5,292 people in the UK died from pulmonary fibrosis (0.9% of all deaths and 4.6% of deaths from lung disease), up from 3,964 in 2008. 

UK deaths from idiopathic pulmonary fibrosis compared with other lung diseases, 2012

How many males and females died from IPF in the UK in 2012?

In 2012, of the 5,292 people who died from pulmonary fibrosis, 3,193 were males and 2,099 were females.

How old were the people who died from IPF in the UK in 2012?

In 2012, of the 5,292 people who died from pulmonary fibrosis:

  • 3 were aged 0-14
  • 475 were aged 15-64
  • 4,814 were aged 65 and above

Standard of living and IPF

Does your standard of living affect your chances of having IPF in the UK?

Figures for 2004-12 show that the proportion of the population ever diagnosed with IPF does not vary much according to the standard of living.

Number of people per 100,000 ever diagnosed with idiopathic pulmonary fibrosis, by standard of living, 2004–12

Find out how the standard of living figures were calculated: Methodology - standard of living