Introduction to idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis is a specific form of chronic, progressive fibrosing lung disease of unknown cause.

It typically occurs in people older than 65 years of age and is more common in males with a history of smoking.

A family history of interstitial lung disease (ILD) (not necessarily only idiopathic pulmonary fibrosis) may be present in around 10% of patients; the exact pattern of inheritance and genes involved is not well understood.

The natural history of idiopathic pulmonary fibrosis is variable and unpredictable. If untreated, progressive deterioration and death generally occur within 3 to 5 years of diagnosis.

Disease progression is characterised by worsening dyspnoea and cough, deteriorating pulmonary function tests and worsening fibrosis on high-resolution computed tomography (HRCT). Antifibrotic drugs (nintedanib, pirfenidone) may slow disease progression; see Treatment of idiopathic pulmonary fibrosis for more information.

Patient and carer information sheets on idiopathic pulmonary fibrosis are available from the Lung Foundation Australia website.