About Pulmonary Fibrosis

(Idiopathic) Pulmonary Fibrosis or 'IPF' is a respiratory disease which affects the lungs and is the most common form of pulmonary fibrosis

‘Idiopathic’ means that the cause of the disease is unknown.

  • What is IPF?

    Pulmonary fibrosis is a scarring disease which affects the lungs. Pulmonary fibrosis is a term that describes the formation of scar tissue within the lungs. It usually occurs around the small air sacs or alveoli of the lungs where it causes thickening and stiffness.

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  • How does it affect the lungs?

    The alveoli are important for the transfer of oxygen and carbon dioxide to and from the lungs and the bloodstream. Over time, the scar tissue builds up around the alveoli, making it harder for oxygen to pass from the lungs to the body where it is needed.

  • What happens next?

    This results in feeling short of breath. At first, this may occur with activities such as walking uphill or stairs. However as the fibrosis increases, you may notice you are breathless with all activities, including washing and dressing, eating and talking.

  • Causes

    IPF usually affects people over the age of 50 years and it is slightly more common in men than women. Studies have demonstrated the IPF is more common in people who smoke or have smoked, and in association with certain occupations where people inhale fibres or wood or metal dust.

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  • Diagnosis

    There are many different types of pulmonary fibrosis and it is important determine what kind of pulmonary fibrosis you have. In order to do this, your specialist will ask about your breathing problems, perform a physical examination, and order a number of tests.

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  • Treatment

    There is no cure for IPF but there are treatments available that may slow down the rate of progression of the lung fibrosis. Nintedanib (Ofev) and pirfenidone (Esbriet) are approved for treating certain people with IPF.

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  • Lung Transplantation

    Lung transplantation is an option for a small number of people with IPF. It is a decision that is not to be taken lightly and needs careful consideration and assessment.

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Action for Pulmonary Fibrosis > About Pulmonary Fibrosis