Idiopathic pulmonary fibrosis (IPF) is a specific and the most common form within a heterogeneous group of idiopathic interstitial pneumonias of unknown aetiology, affecting the lung parenchyma. IPF is a chronic, progressive and debilitating disease, characterised by inflammation and scarring of lung tissue resulting in loss of lung function and impaired gas exchange over time.1,2
Generally, IPF is more common in men and it tends to develop later in life.3 A number of other patient-related and environmental risk factors have been proposed (see table below), although the precise aetiology of the disease is unknown.
There are limited treatment options available for IPF and the clinical course is variable. Most patients face a gradual deterioration, although some may progress rapidly or be subject to episodes of rapid deterioration, known as acute exacerbations.6,7
Overall prognosis in IPF patients is poor, with a median survival of 2-3 years after diagnosis.1 This survival rate is even lower than that of several common cancers (see graphic below).8
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