Idiopathic Pulmonary Fibrosis

What Is Idiopathic Pulmonary Fibrosis?

Idiopathic pulmonary fibrosis (IPF) is a serious condition that causes the tiny air sacs in the lungs (known as the alveoli) and the surrounding tissue to become damaged. As the condition progresses and gets worse the tissue also becomes scarred. The fact that the condition is known as idiopathic infers that the exact cause is unknown.

IPF used to be known as Cryptogenic Fibrosing Alveolitis. Cryptogenic and idiopathic both mean of unknown cause, while fibrosis means thickening and scarring. Idiopathic pulmonary fibrosis is a rare disease and affects fewer than 3 in 10,000 people in the UK and is most common among people aged between 50 and 70.

What causes idiopathic pulmonary fibrosis?

The exact cause of idiopathic pulmonary fibrosis is not known. It was believed that inflammation was the cause of the condition, but treatments designed to ease swelling are not always effective at treating IPF and therefore the role of inflammation has come under scrutiny.

Experts now think that IPF may be caused by damage to the cells in the inner lining of the alveoli. As a result of this damage, the cells try to heal themselves but the process is abnormal and causes the cells to become more damaged and the lining of the alveoli and lung tissue to become scarred. The scarring and thickening of the cells obstructs the flow of oxygen through the blood vessels, which means less oxygen is taken into the body when you breathe in.

The following triggers for IPF have been identified:

• Smoking.
• Certain forms of medication.
• Environmental factors, including inhaling certain pollutants.
• Viral infections.
• Gastro-oesophageal reflux.

What are the symptoms of idiopathic pulmonary fibrosis?

Symptoms of idiopathic pulmonary fibrosis tend to develop slowly and include:

• Breathlessness, which tends to get worse as the disease progresses.
• A dry cough.
• Tiredness.
• Increased susceptibility to chest infections.
• Finger and toenail clubbing: this is when the base of the nails becomes swollen.

How is idiopathic pulmonary fibrosis diagnosed?

The presence of the symptoms listed above, coupled with listening to the chest and hearing crackles, is usually sufficient for doctors to diagnose IPF. However, a CT scan, biopsy test and lung function tests will be conducted to confirm the diagnosis. A high resolution CT scan is commonly used in the diagnosis of IPF. If your doctor suspects that you may have developed heart failure, you may be advised to have an echocardiogram, which is an ultrasound scan of your heart.

Treatment for idiopathic pulmonary fibrosis

There is currently no cure for idiopathic pulmonary fibrosis, however, treatments are available to suppress and ease symptoms.
Treatments in the past have been focused on reducing inflammation, as this was thought to be the primary cause of IPF. These treatments are still used but research into new drugs is being carried out, as existing medications are not effective in some patients. Treatments include:

• Steroids: research suggests steroids are effective in around 1 in 4 people.
• Immunosuppressant drugs: these drugs help to reduce inflammation but they are only effective in some cases; a combination of steroids and immunosuppressants may also be used.
• No treatment: in some cases, doctors will adopt a policy of watchful waiting, as medication can cause unpleasant side-effects. This is most common among elderly patients when symptoms are developing very slowly.
• Oxygen treatment: this is only used when symptoms are very severe.
• Lung transplant: this is usually a last resort and is most commonly performed on young people who suffer with severe symptoms.

Self-help

There are some steps you can take to try and ease your symptoms, including:

• Stop smoking.
• Have a flu and pneumococcal vaccination every autumn.

What is the outlook for idiopathic pulmonary fibrosis?

The outlook varies greatly according to the individual. Some people remain stable for many years after diagnosis, while others develop severe symptoms and die within a few years of diagnosis. Lung transplants have been found to be successful in young people, but the availability of donors is often a problem.