Pulmonary fibrosis (idiopathic)
Idiopathic pulmonary fibrosis (IPF) is a rare and poorly understood lung condition that causes scarring of the lungs.
It gets worse over time and is often fatal.
Symptoms of IPF 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 be connected with cells inside the lungs known as alveolar epithelial cells (AECs).
The current thinking is that the AECs become damaged and then 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.
Levels of fibrosis increase leading to a gradual decline in lung function resulting in the symptoms of IPF.
Exactly why the AECs become damaged in the first place is not known. A combination of genetic and environmental factors may be involved.
Read more about the possible causes of idiopathic pulmonary fibrosis.
There is currently no cure for IPF so the aim of treatment is to try to relieve symptoms and protect against anything that could further damage the lungs such as an influenza (flu) infection.
This can be done using a combination of medication and oxygen therapy (using a breathing mask and tank).
In some cases treatment might not be used, as side effects associated with some types of medication outweigh potential benefits.
Younger people with IPF who are in a relatively good state of health may be a suitable candidate for a lung transplant.
Read more about the treatment of idiopathic pulmonary fibrosis.
Who is affected
IPF is a rare condition though the number of cases have risen in recent years. It is estimated that for every 100,000 people in England only four will be newly diagnosed with IPF in any given year.
The condition usually first develops in adults aged 50 or above and is thought to be more common in men.
The outlook for IPF can be highly variable. Some people respond well to medication 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. In general, survival times can range from 2-5 years after diagnosis.