What is pulmonary fibrosis?

Pulmonary fibrosis is a lung disease. Sometimes pulmonary fibrosis is called 'interstitial lung disease'.

In this condition, the tissue around the air sacs in your lungs — known as alveoli — becomes damaged, thickened and scarred. As your lungs scar and stiffen, breathing becomes more difficult.

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 cough that does not go away
• being tired
• losing weight
• losing your appetite
• having bulging finger or toe tips, known as clubbing

Pulmonary fibrosis symptoms tend to get worse with time.

What causes pulmonary fibrosis?

The cause of pulmonary fibrosis is often not known. This is called 'idiopathic pulmonary fibrosis'.

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

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

Your chance of developing pulmonary fibrosis is higher if you:

• are over 60 years old
• smoke or used to smoke
• work or live in a place 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 clubbed fingernails or toenails
• have symptoms that are not improving or getting worse

When to seek urgent care

If your symptoms suddenly get worse or you are having trouble breathing, call triple zero (000) for an ambulance.

How is pulmonary fibrosis diagnosed?

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

They will ask about:

• your work
• if you smoke
• your general health
• your family history
• the medicines you take

Your doctor may recommend tests such as:

• blood tests
• a chest x-ray
• a CT 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

If your doctor thinks you have pulmonary fibrosis, they will refer you to a doctor who specialises in lung conditions.

You may also see a specialist nurse, who can help you manage your condition.

How is pulmonary fibrosis treated?

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

• stop or slow down the disease
• help improve your symptoms
• help improve your quality of life

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

Medicines and oxygen for pulmonary fibrosis

Antifibrotic medicines are available. These medicines can slow down the disease in some people. There are 2 medicines currently available:

1. nintedanib
2. pirfenidone

Talk with your doctor about the side effects of these medicines and whether they may be suitable for you.

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

Pulmonary rehabilitation

Many people benefit from pulmonary rehabilitation, which is a program that includes:

• exercise training
• information and education, such as dietary advice and stress management tips
• advice to help manage breathlessness

Other treatment options

If you smoke, you should quit smoking to help with your symptoms. It will also reduce your lung damage.

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

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

Living with pulmonary fibrosis

It's important to try to keep up your social connections and continue with activities you enjoy as much as possible.

Talk to your doctor, trusted friends or family members if you need support.

There are also support groups for people with pulmonary fibrosis.

Your doctor may also suggest you see a psychologist for support. They can help if you have emotional distress or develop anxiety or depression.

Talk to your healthcare team if you are planning on travelling, especially if by plane. You may need extra vaccinations or oxygen for the trip.

What are the complications of pulmonary fibrosis?

Having idiopathic pulmonary fibrosis can increase your chance of developing:

• lung cancer
• pulmonary hypertension — higher than normal blood pressure in the arteries that carry blood from your heart to your lungs
• lung infections
• blood clots, including deep vein thrombosis (DVT) and pulmonary embolism
• anxiety
• depression

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

Can pulmonary fibrosis be prevented?

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

• quitting smoking
• avoiding known causes and risk factors where possible

You can also take steps to be as healthy as possible with pulmonary fibrosis, including:

• eating a healthy diet
• exercising and staying physically active
• getting treatment if you have a chest infection

It's important to get vaccinated against the flu every year. Other vaccines may also be recommended, such as COVID-19 and pneumococcal disease vaccines. This can help prevent complications from chest infections.

Talk with your doctor about vaccinations.

Resources and support

Lung Foundation Australia has information and support services for people with pulmonary fibrosis. They have information in languages other than English. You can call their Information and Support Centre on 1800 654 301.

The Centre of Research Excellence in Pulmonary Fibrosis has information and support for people with pulmonary fibrosis.

You can also 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.