Progressive pulmonary fibrosis: a state-of-the-art review

Fibrosing interstitial lung disease encompasses a large number of diverse conditions, the prototype being idiopathic pulmonary fibrosis (IPF). IPF is characterised by irreversible progression and accounts for loss of lung function, exercise intolerance and complications, especially acute exacerbation and respiratory failure leading to early mortality. A significant proportion of patients with fibr…