@article{JTD4140,
author = {Maya M. Juarez and Andrew L. Chan and Andrew G. Norris and Brian M. Morrissey and Timothy E. Albertson},
title = {Acute exacerbation of idiopathic pulmonary fibrosis—a review of current and novel pharmacotherapies},
journal = {Journal of Thoracic Disease},
volume = {7},
number = {3},
year = {2015},
keywords = {},
abstract = {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.},
issn = {2077-6624}, url = {https://jtd.amegroups.org/article/view/4140}
}