Idiopathic pulmonary fibrosis (IPF) is a rare but inevitably progressive and fatal lung disease, with a prognosis that can be worse than many forms of cancer [1, 2]. There have been a number of important advances and achievements in this field over the past 2 years, which have increased our understanding of how IPF can be managed and brought new hope for patients who have this devastating disease. Until recently, there were no pharmacological treatments approved for patients with IPF in Europe. A major advance in 2011 was the European approval of pirfenidone for adults with mild-to-moderate IPF. Since its introduction, there is now a growing body of evidence regarding the use of pirfenidone in clinical practice, enabling clinicians to discuss some key issues regarding the recommended management of IPF, including the role of earlier diagnosis of IPF, expected clinical outcomes with pirfenidone in the real-world setting, and the role of the multidisciplinary team in diagnosis.
With this background of increasing knowledge and clinical experience in the management of IPF, this issue of the European Respiratory Review includes two articles highlighting the substantial progress that has been made in the management of IPF. These articles are based on presentations from a symposium held at the 2013 European Respiratory Society Annual Congress entitled “Evolvement of best practice in diagnosis and management of IPF”. Cottin and Richeldi [3] review the neglected evidence in IPF including the role of earlier diagnosis and an update on an ongoing clinical study investigating correlations between lung sounds on auscultation and high-resolution computed tomography images. Kreuter [4] introduces new data analyses regarding pirfenidone, including a per-protocol analysis of the CAPACITY studies, new data from the RECAP extension study and expert centre experience. These articles provide an excellent opportunity for all clinicians involved in the care of patients with IPF to review key concepts and the evolving role of pirfenidone treatment in the management of this disease.
Supplementary Material
Acknowledgments
This article is based on the proceedings of a satellite symposium held at the 2013 European Respiratory Society Annual Congress (Barcelona, Spain), which was sponsored by InterMune. I was assisted in the preparation of the article by IntraMed International (Milan, Italy), funded by InterMune.
Footnotes
Provenance: Publication of this peer-reviewed article was supported by InterMune International AG, Muttenz, Switzerland (article sponsor, European Respiratory Review issue 131).
Statement of Interest: Disclosures can be found alongside the online version of this article at err.ersjournals.com
References
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