Abstract
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.
Keywords: Idiopathic pulmonary fibrosis, interstitial lung diseases, new treatments, nintedanib, pathogenesis, pirfenidoen.
Graphical Abstract
57
2
2