title: The treatment of idiopathic pulmonary fibrosis.
abstract: Idiopathic pulmonary fibrosis (IPF) is a progressive and invariably fatal disease with a median survival of less than three years from diagnosis. The last decade has seen an exponential increase in clinical trial activity in IPF and this in turn has led to important developments in the treatment of this terrible disease. Previous therapeutic approaches based around regimens including corticosteroids and azathioprine have, when tested in randomized clinical trials, been shown to be harmful in IPF. By contrast, compounds with anti-fibrotic actions have been shown to be beneficial. Subsequently, the novel anti-fibrotic agent pirfenidone has, in many parts of the world, become the first treatment ever to be licensed for use in IPF. This exciting development, coupled with ongoing clinical trials of a range of other novel compounds, is bringing hope to patients and their clinicians and raises the prospect that, in the future, it may become possible to successfully arrest the development of progressive scarring in IPF.
authors.affiliation: NIHR Respiratory Biomedical Research Unit, Royal Brompton Hospital Sydney Street, London, SW3 6NP UK ; Centre for Inflammation and Tissue Repair, University College London, Rayne Institute 5 University Road, London, WC1E 6JJ UK.
authors.forename: Hannah V
authors.initials: HV
authors.lastname: Woodcock
authors.affiliation: NIHR Respiratory Biomedical Research Unit, Royal Brompton Hospital Sydney Street, London, SW3 6NP UK ; Centre for Leucocyte Biology, Imperial College London Sir Alexander Fleming Building, London, SW3 UK.
authors.forename: Toby M
authors.initials: TM
authors.lastname: Maher