TY - JOUR AU - Juarez, Maya M. AU - Chan, Andrew L. AU - Norris, Andrew G. AU - Morrissey, Brian M. AU - Albertson, Timothy E. PY - 2015 TI - Acute exacerbation of idiopathic pulmonary fibrosis—a review of current and novel pharmacotherapies JF - Journal of Thoracic Disease; Vol 7, No 3 (March 30, 2015): Journal of Thoracic Disease Y2 - 2015 KW - N2 - 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. UR - https://jtd.amegroups.org/article/view/4140