What is pulmonary fibrosis?

Pulmonary fibrosis is a lung disease where the lace-like 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 and not enough oxygen is able to enter the bloodstream.

What are the symptoms of pulmonary fibrosis?

The symptoms of pulmonary fibrosis may include:

• being short of breath — at first only when active, but then later when you are 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

Unless something can be done to treat the underlying cause, pulmonary fibrosis tends to get worse.

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. Such causes include:

• some medicines used in radiotherapy treatment or to treat seizures, heart problems or infections
• breathing in harmful dust or chemicals at a workplace or in the environment, such as mould, asbestos, silica, metal dusts and toxic fumes
• certain medications, such as chemotherapy medications and antibiotics, as well as recreational drugs
• having an autoimmune disease such as rheumatoid arthritis or a disease of the connective tissue
• having the inflammatory disease sarcoidosis

Risk factors for pulmonary fibrosis

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

• are over 60
• smoke or used to smoke
• live on a farm
• work in the agriculture or livestock sector
• work or live in an environment where you breathe in smoke or dust from wood, metal, stone or sand
• have pulmonary fibrosis in the family

How is pulmonary fibrosis diagnosed?

If your doctor suspects you might have pulmonary fibrosis, they will talk to you and examine you, and will ask about your work, smoking and anything else that could affect your lungs. They may order tests such as:

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

How is pulmonary fibrosis treated?

If you are diagnosed with pulmonary fibrosis, you will most likely be treated by a multidisciplinary team. Your treatment could include:

• medications such as steroids to prevent permanent lung changes, prevent scarring, reduce inflammation or dampen down the body’s immune system
• puffers or oxygen therapy to ease breathing
• a lung transplant if the pulmonary fibrosis is severe

Many people benefit from pulmonary rehabilitation, a program that includes exercise, information and advice to help manage breathlessness. These programs are run by a physiotherapist, occupational therapist or other allied health professional.

It is also important to see a psychologist or counsellor if you are feeling anxious or depressed.

Treating any underlying cause can help. For example, people with drug-induced pulmonary fibrosis should stop using the drug.

For people with severe pulmonary fibrosis, palliative care to control the symptoms can improve their quality of life.