Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with a devastating prognosis. The pathogenesis is not clear but involves repetitive lung injury with aberrant inflammatory response. In the last two decades, multiple therapeutic drug trials have been completed failing to identify an effective treatment for this disease. Lung transplant has been the only effective option for patients with IPF. Over the last 5 years, two agents have demonstrated significant slowing in IPF progression. Pirfenidone and nintedanibe are new drugs that showed promising results in IPF treatment. The drugs were approved in Japan and Europe in 2008 and 2011 respectively. The U.S Food and Drugs Administrations approved these medications recently in October 2014. Published clinical trials are reviewed with these two agents and the risks and benefits of therapy for IPF are discussed. Information is provided in this review to assist physicians in the decision making process related to starting one of these agents in patients with IPF.
机构:
Mayo Clin, Dept Pulm & Crit Care Med, Gonda 18 South, Rochester, MN 55905 USAMayo Clin, Dept Pulm & Crit Care Med, Gonda 18 South, Rochester, MN 55905 USA
Dempsey, Timothy M.
Sangaralingham, Lindsey R.
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Mayo Clin, Robert D & Patricia E Kern Ctr Sci Hlth Care Deli, Rochester, MN 55905 USA
OptumLabs, Cambridge, MA USAMayo Clin, Dept Pulm & Crit Care Med, Gonda 18 South, Rochester, MN 55905 USA
Sangaralingham, Lindsey R.
Yao, Xiaoxi
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Mayo Clin, Dept Hlth Sci Res, Rochester, MN 55905 USAMayo Clin, Dept Pulm & Crit Care Med, Gonda 18 South, Rochester, MN 55905 USA
Yao, Xiaoxi
Sanghavi, Darshak
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OptumLabs, Cambridge, MA USAMayo Clin, Dept Pulm & Crit Care Med, Gonda 18 South, Rochester, MN 55905 USA
Sanghavi, Darshak
Shah, Nilay D.
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Mayo Clin, Robert D & Patricia E Kern Ctr Sci Hlth Care Deli, Rochester, MN 55905 USA
Mayo Clin, Dept Hlth Sci Res, Rochester, MN 55905 USAMayo Clin, Dept Pulm & Crit Care Med, Gonda 18 South, Rochester, MN 55905 USA
Shah, Nilay D.
Limper, Andrew H.
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Mayo Clin, Dept Pulm & Crit Care Med, Gonda 18 South, Rochester, MN 55905 USA
Mayo Clin, Robert D & Patricia E Kern Ctr Sci Hlth Care Deli, Rochester, MN 55905 USAMayo Clin, Dept Pulm & Crit Care Med, Gonda 18 South, Rochester, MN 55905 USA
机构:
Columbia Univ, Irving Med Ctr, Dept Med, New York, NY USA
Columbia Univ, Irving Med Ctr, Dept Epidemiol, New York, NY USAHeidelberg Univ, Thoraxklin, Ctr Interstitial & Rare Lung Dis, Pneumonol, Heidelberg, Germany
Lederer, David J.
Cottin, Vincent
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机构:
Claude Bernard Univ Lyon 1, Louis Pradel Hosp, Reference Ctr Rare Pulm Dis, Dept Resp Med,UMR754, Lyon, FranceHeidelberg Univ, Thoraxklin, Ctr Interstitial & Rare Lung Dis, Pneumonol, Heidelberg, Germany
机构:
Univ North Texas, Denton, TX 76203 USA
Texas A&M Univ, College Stn, TX 77843 USA
Corpus Christi Med Ctr, Corpus Christi, TX USAUniv North Texas, Denton, TX 76203 USA