What is pulmonary fibrosis?

Pulmonary fibrosis is a lung disease. The tissue around the air sacs of the lungs — known as alveoli — becomes damaged, thickened, and scarred.

As the lungs scar and stiffen, breathing becomes more difficult. It can mean that not enough oxygen enters your blood.

What are the symptoms of pulmonary fibrosis?

The symptoms of pulmonary fibrosis can include:

• being short of breath — at first only when you’re active, but then later when you’re just resting
• a dry, hacking cough that does not go away
• being tired
• losing weight
• losing your appetite
• having bulging finger or toe tips, known as clubbing
• aching joints and muscles

Pulmonary fibrosis symptoms tend to get worse with time.

What causes pulmonary fibrosis?

Most often, the cause of pulmonary fibrosis is not known. If this is the case, it is called idiopathic pulmonary fibrosis (idiopathic means the cause is unknown).

In some people, a cause can be found. Some causes include:

• certain medicines
• having radiation treatment in the past
• breathing in harmful dust or chemicals at a workplace or in the environment, like asbestos, silica, metal dusts, and coal dust
• having an autoimmune disease like rheumatoid arthritis or a connective tissue disease
• having certain inflammatory conditions, such as sarcoidosis

You are more at risk than others of developing pulmonary fibrosis if you:

• are over 60 years old
• smoke or used to smoke
• work or live in an environment where you breathe in smoke or dust from wood, metal, stone, coal or sand
• have family members who have pulmonary fibrosis

When should I see my doctor?

You should see your doctor if you:

• are short of breath
• have a dry cough that doesn’t get better
• often feel tired
• are losing weight without trying, or have lost your appetite
• have blue lips and tongue
• have clubbed fingernails or toenails
• have symptoms that are not improving, or they are getting worse

How is pulmonary fibrosis diagnosed?

Your doctor will talk with you about your symptoms and examine you.

They may ask about your work, smoking habits, and anything else that could affect your lungs.

Your doctor may recommend tests such as:

• blood tests
• chest x-ray
• CT scan or an MRI scan
• lung function tests
• a bronchoscopy (a procedure to look inside your lungs)
• a lung biopsy, where a small sample of your lung tissue is taken to be examined in the laboratory

How is pulmonary fibrosis treated?

There is no cure for pulmonary fibrosis, but there are several treatments available. Treatments can:

• stop or slow the progression of the disease
• help improve symptoms

Treatment will depend on many things, including the cause of your pulmonary fibrosis.

Medicines and oxygen

Antifibrotic medicines are available that can slow down idiopathic pulmonary fibrosis in those with mild or moderate disease. There are 2 medicines currently available:

• nintedanib
• pirfenidone

Medicines to reduce inflammation may be helpful for some people. Medicines to control breathlessness and oxygen may also help.

Pulmonary rehabilitation

Many people benefit from pulmonary rehabilitation, which is a program that includes exercise, information, and advice to help manage breathlessness.

Other treatments

A lung transplant may be recommended for some people with severe pulmonary fibrosis.

For people with severe pulmonary fibrosis, palliative care can help control symptoms and improve quality of life.

Can pulmonary fibrosis be prevented?

Things you can do which may help prevent pulmonary fibrosis include:

• quitting smoking
• eating a healthy diet
• exercising and staying physically active
• getting treatment if you have a chest infection
• avoiding known causes and risk factors where possible

Complications of pulmonary fibrosis

Having idiopathic pulmonary fibrosis can increase your chance of developing:

• lung cancer
• pulmonary hypertension, where the blood pressure in the arteries that carry blood from the heart to the lungs is higher than normal
• lung infections
• anxiety
• depression

Talk to your doctor if you are concerned about complications or need support.

Resources and support

Lung Foundation Australia has information and support services for people with lung conditions. You can call their Information and Support Centre on 1800 654 301.

You can call the healthdirect helpline on 1800 022 222 (known as NURSE-ON-CALL in Victoria). A registered nurse is available to speak with 24 hours a day, 7 days a week.