Pulmonary fibrosis is the term used to describe a range of rare conditions where the lungs become scarred. As the scar tissue in a person’s lungs builds up and thickens, it progressively affects their breathing.
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 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 meaning the cause is unknown).
In some people, a cause can be found. Such causes include:
- radiotherapy treatment
- breathing in harmful dust at a workplace or in the environment
- certain medications, such as chemotherapy medications and antibiotics, as well as recreational drugs
- genetic factors
- bird or animal droppings
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
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
- having bulging finger or toe tips, known as clubbing
Unless something can be done to treat the underlying cause, pulmonary fibrosis tends to get worse.
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:
You might see a specialist, and you might have a test where the specialist looks inside your lungs and removes a sample of lung tissue or cells for testing. This can be done under light sedation or under a general anaesthetic.
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:
- medicines, such as
- tablets that slow the development of scarring
- tablets to control inflammation
- puffers to ease your breathing
- oxygen therapy to assist breathing
- a lung transplant if the pulmonary fibrosis is really severe
Many people benefit from pulmonary rehabilitation, a program that includes exercise, information and advice. These programs are run by a physiotherapist, occupational therapist or other allied health professional.
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.
Last reviewed: February 2019