1. Acute exacerbation of idiopathic pulmonary fibrosis-a review of current and novel pharmacotherapies
- Author
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Juarez, Maya M, Chan, Andrew L, Norris, Andrew G, Morrissey, Brian M, and Albertson, Timothy E
- Subjects
clinical trials ,Orphan Drug ,Rare Diseases ,treatment ,Prevention ,Clinical Sciences ,Respiratory ,Idiopathic pulmonary fibrosis ,Autoimmune Disease ,Lung ,acute exacerbation ,drug therapy - 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.
- Published
- 2015