%0 Journal Article %T Acute exacerbation of idiopathic pulmonary fibrosis—a review of current and novel pharmacotherapies %A Juarez, Maya M. %A Chan, Andrew L. %A Norris, Andrew G. %A Morrissey, Brian M. %A Albertson, Timothy E. %J Journal of Thoracic Disease %D 2015 %B 2015 %9 %! Acute exacerbation of idiopathic pulmonary fibrosis—a review of current and novel pharmacotherapies %K %X Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive form of lung disease of unknown etiology for which a paucity of therapies suggest benefit, and for which none have demonstrated improved survival. Acute exacerbation of IPF (AE-IPF) is defined as a sudden acceleration of the disease or an idiopathic acute injury superimposed on diseased lung that leads to a significant decline in lung function. An AE-IPF is associated with a mortality rate as high as 85% with mean survival periods of between 3 to 13 days. Under these circumstances, mechanical ventilation (MV) is controversial, unless used a as a bridge to lung transplantation. Judicious fluid management may be helpful. Pharmaceutical treatment regimens for AE-IPF include the use of high dose corticosteroids with or without immunosuppressive agents such as cyclosporine A (CsA) , and broad spectrum antibiotics, despite the lack of convincing evidence demonstrating benefit. Newer research focuses on abnormal wound healing as a cause of fibrosis and preventing fibrosis itself through blocking growth factors and their downstream intra-cellular signaling pathways. Several novel pharmaceutical approaches are discussed. %U https://jtd.amegroups.org/article/view/4140 %V 7 %N 3 %P 499-519 %@ 2077-6624