Therapeutic advances in idiopathic pulmonary fibrosis

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...

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Published inClinical medicine (London, England) Vol. 16; no. 1; pp. 42 - 51
Main Authors Fraser, Emily, Hoyles, Rachel K
Format Journal Article
LanguageEnglish
Published England Elsevier Ltd 01.02.2016
Royal College of Physicians
Subjects
anti-fibrotic agents
Anti-Inflammatory Agents - therapeutic use
Drug Therapies In
Humans
idiopathic pulmonary fibrosis
Idiopathic Pulmonary Fibrosis - diagnosis
Idiopathic Pulmonary Fibrosis - physiopathology
Idiopathic Pulmonary Fibrosis - therapy
Indoles - therapeutic use
IPF
nintedanib
pirfenidone
Pyridones - therapeutic use
nintedanib
idiopathic pulmonary fibrosis
anti-fibrotic agents
pirfenidone
IPF
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Summary: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.
Bibliography:SourceType-Scholarly Journals-1
ObjectType-General Information-1
content type line 14
ISSN:1470-2118
1473-4893
DOI:10.7861/clinmedicine.16-1-42