Pulmonary fibrosis (idiopathic)

Idiopathic pulmonary fibrosis (IPF) is a rare and poorly understood lung condition that causes scarring of the lungs. 

Idiopathic means the cause of the condition is not known.

IPF gets worse over time and is often fatal.

Symptoms include:

• shortness of breath – especially when physically active – which gradually worsens over time
• a persistent dry cough

Read more about the symptoms of idiopathic pulmonary fibrosis.

What causes pulmonary fibrosis?

The cause of IPF is still unclear, but the condition appears to involve the cells that line the tiny air sacs in the lungs (alveoli). These are called alveolar epithelial cells (AECs).

The AECs appear to become damaged and begin to die. The body tries to repair the damage by releasing another type of cell known as fibroblasts. But the production of the fibroblasts goes out of control and they cause scarring and hardening (fibrosis) of the delicate tissues of the lungs.

As this scarring gets worse the lungs find it more difficult to work properly, resulting in the symptoms of IPF.

But exactly why the AECs become damaged in the first place isn't clear. A combination of genetic and environmental factors may be involved.

Read more about the possible causes of idiopathic pulmonary fibrosis.

Treatment

There is currently no cure for IPF so the aim of treatment is to try to relieve symptoms and slow its progression.

Breathing oxygen through a mask and pulmonary rehabilitation – exercises and advice to help you breathe more easily – may be recommended to treat breathlessness. This is based on an assessment if you are diagnosed with IPF.

A medication called pirfenidone may be used for treating adults with IPF who have a lung capacity of 50-80% of the expected value.

Younger people with IPF who are in a relatively good state of health may be suitable candidates for a lung transplant.

Read more about the treatment of idiopathic pulmonary fibrosis.

Who is affected

IPF is a relatively rare condition, although the number of cases has risen in recent years. It is estimated that more than 5,000 cases are diagnosed every year in the UK.

The condition usually first develops in adults aged 50 or above and is thought to be more common in men.

Outlook

The outlook for IPF can be highly variable. Some people respond well to treatment and remain relatively free of symptoms for many years.

Other people's health quickly worsens leading to a fatal complication, such as heart failure.

It is impossible to predict an individual’s expected survival rate as the rate of progression can vary greatly.

In the UK, the average survival for people with IPF is three years from the time of diagnosis. However, around 20% of people with the disease survive for more than five years.