Pharmacological Treatment of Idiopathic Pulmonary Fibrosis – An Update

Paolo Spagnolo,1 Hilario Nunes,2 Wim A. Wuyts3

1. Centre for Rare Lung Diseases, Respiratory Disease Unit, Department of Medical and Surgical Sciences for Children and Adults, University Hospital of Modena, Italy
2. Assistance Publique, Hôpitaux de Paris, Service de Pneumologie, Hôpital Avicenne, Bobigny, Université Paris 13, Sorbonne Paris, France
3. Unit for Interstitial Lung Diseases, Department of Respiratory Medicine, University Hospitals Leuven, Belgium

Disclosure: No potential conflict of interest.
Received: 27.06.2013 Accepted: 05.09.2013
Citation: EMJ Respir. 2013;1:108-121.


Idiopathic pulmonary fibrosis (IPF) is the most common and most lethal fibrosing interstitial pneumonia, with a mortality rate that exceeds that of many cancers. Currently, there is no standard treatment recommended by the guidelines. A number of high-quality clinical trials evaluating novel potential therapies have recently been concluded. While the results have mostly been disappointing, some compounds appear promising in reducing disease progression. In this regard, pirfenidone is the most advanced molecule for IPF treatment, having been approved in Europe, Japan, India, and Canada. However, due to the complexity and uncertainties intrinsic to IPF, it is essential that each therapeutic strategy be tailored to the individual patient, after evaluation of potential benefits and pitfalls. Randomised controlled trials represent a valid choice for IPF patients. Many agents with high potential are being tested and many more are ready to be tested in clinical trials. Their completion is critically important to achieve the ultimate goal of curing IPF.

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