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Pharmacological Treatment of Idiopathic Pulmonary Fibrosis

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Idiopathic Pulmonary Fibrosis

Part of the book series: Respiratory Medicine ((RM,volume 9))

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Abstract

Idiopathic pulmonary fibrosis (IPF) is the most common and most lethal diffuse fibrosing lung disease, with a mortality rate that exceeds that of many types of cancer. At present there is no effective standard treatment recommended by guideline documents. However, several high-quality clinical trials evaluating a number of novel therapies have recently been concluded. The results have mostly been disappointing, although some compounds have shown promising results. In particular, pirfenidone seems to be the most advanced agent for IPF treatment, having been approved in Europe, Japan, and India. In general, due to the complexity and the uncertainties intrinsic to IPF, it is essential that each therapeutic strategy be tailored to the individual patient after discussing the potential benefits and pitfalls. Randomized controlled trials still represent a valid choice for IPF patients, and their completion is critically important to achieving the ultimate goal of curing IPF.

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Author information

Authors and Affiliations

  1. Center for Rare Lung Diseases, University Hospital Policlinico of Modena, Modena, Italy

    Paolo Spagnolo M.D., Ph.D., Fabrizio Luppi M.D., Ph.D. & Gloria Montanari M.D.

  2. Chair of Interstitial Lung Disease, University Hospital Southampton, Tremona Road, Southampton, SO16 6YD, UK

    Luca Richeldi M.D., Ph.D.

Authors
  1. Paolo Spagnolo M.D., Ph.D.
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  2. Fabrizio Luppi M.D., Ph.D.
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  3. Gloria Montanari M.D.
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  4. Luca Richeldi M.D., Ph.D.
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Corresponding author

Correspondence to Luca Richeldi M.D., Ph.D. .

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Editors and Affiliations

  1. Dept. Internal Medicine, Sect. Allergy Pulmonary & Critical Care Med., University of Wisconsin School of Medicine & Public Health, Madison, Wisconsin, USA

    Keith C. Meyer

  2. Advanced Lung Disease and Lung Transplant Program, Inova Fairfax Hospital, Falls Church, Virginia, USA

    Steven D. Nathan

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Spagnolo, P., Luppi, F., Montanari, G., Richeldi, L. (2014). Pharmacological Treatment of Idiopathic Pulmonary Fibrosis. In: Meyer, K., Nathan, S. (eds) Idiopathic Pulmonary Fibrosis. Respiratory Medicine, vol 9. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-62703-682-5_14

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  • DOI: https://doi.org/10.1007/978-1-62703-682-5_14

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