Idiopathic Pulmonary Fibrosis (IPF): An Overview
Shaney L. Barratt 1,2,*, Andrew Creamer 1, Conal Hayton 3 and Nazia Chaudhuri 3
1 Bristol Interstitial Lung Disease Service, North Bristol NHS Trust, Bristol BS10 5NB, UK;
andrew.creamer@nbt.nhs.uk
2 Academic Respiratory Unit, University of Bristol, Bristol BS16 1QY, UK
3 North West Interstitial Lung Disease Unit, Manchester University NHS Foundation Trust, Wythenshawe,
Manchester M23 9LT, UK; conal.hayton@nhs.net (C.H.); nazia.chaudhuri@nhs.net (N.C.)Correspondence: shaney.barratt@nbt.nhs.uk
Published: 6 August 2018
Abstract: Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease characterised by chronic,
progressive scarring of the lungs and the pathological hallmark of usual interstitial pneumonia.
Current paradigms suggest alveolar epithelial cell damage is a key initiating factor. Globally, incidence
of the disease is rising, with associated high morbidity, mortality, and economic healthcare burden.
Diagnosis relies on a multidisciplinary team approach with exclusion of other causes of interstitial
lung disease. Over recent years, two novel antifibrotic therapies, pirfenidone and nintedanib, have
been developed, providing treatment options for many patients with IPF, with several other agents in
early clinical trials. Current efforts are directed at identifying key biomarkers that may direct more
customized patient-centred healthcare to improve outcomes for these patients in the future.
Keywords: idiopathic pulmonary fibrosis; interstitial lung disease; nintedanib; pirfenidone
© 2018 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access
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Citation
MDPI and ACS Style
Barratt, S.L.; Creamer, A.; Hayton, C.; Chaudhuri, N. Idiopathic Pulmonary Fibrosis (IPF): An Overview. J. Clin. Med. 2018, 7, 201. https://doi.org/10.3390/jcm7080201
AMA Style
Barratt SL, Creamer A, Hayton C, Chaudhuri N. Idiopathic Pulmonary Fibrosis (IPF): An Overview. Journal of Clinical Medicine. 2018; 7(8):201. https://doi.org/10.3390/jcm7080201
Chicago/Turabian Style
Barratt, Shaney L., Andrew Creamer, Conal Hayton, and Nazia Chaudhuri. 2018. “Idiopathic Pulmonary Fibrosis (IPF): An Overview” Journal of Clinical Medicine 7, no. 8: 201. https://doi.org/10.3390/jcm7080201
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