
Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal interstitial lung disease with a median survival of 3–5 years. Its disease course is highly variable, as some patients experience rapid deterioration in lung function while others experience more gradual decline. The development of acute exacerbation of IPF (AE-IPF), a highly lethal complication of unknown aetiology, has been shown to accelerate disease progression.
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