title: Therapeutic advances in idiopathic pulmonary fibrosis.
abstract: Idiopathic pulmonary fibrosis (IPF) is characterised by progressive accumulation of scar tissue in the lung and is associated with a median life expectancy of 2-4 years. Until recently, treatment options were limited, focusing on ineffective anti-inflammatory therapy, palliation, transplant or trial recruitment. Significant recent advances in the field have led to two novel anti-fibrotic agents, pirfenidone and nintedanib, which have been shown to significantly slow disease progression in IPF. This article outlines the approach to management of IPF, the role of specialist centres and specialist interstitial lung disease multidisciplinary review, and explores both the trial evidence and practical considerations in the use of these anti-fibrotic agents.
authors.affiliation: Oxford Interstitial Lung Disease Service, Churchill Hospital, Oxford, UK.
authors.forename: Emily
authors.initials: E
authors.lastname: Fraser
authors.affiliation: Oxford Interstitial Lung Disease Service, Churchill Hospital, Oxford, UK rachel.hoyles@ouh.nhs.uk.
authors.forename: Rachel K
authors.initials: RK
authors.lastname: Hoyles
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis* / Q000503:physiopathology* / Q000628:therapy; D007211:Indoles / Q000627:therapeutic use; D011728:Pyridones / Q000627:therapeutic use
chemical_list: D000893:Anti-Inflammatory Agents; D007211:Indoles; D011728:Pyridones; C093844:pirfenidone; C530716:nintedanib
keywords: IPF; anti-fibrotic agents; idiopathic pulmonary fibrosis; nintedanib; pirfenidone
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