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Acute exacerbation of idiopathic pulmonary fibrosis-a review of current and novel pharmacotherapies.
J Thorac Dis 2015; 7(3):499-519JT

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.

Authors+Show Affiliations

Division of Pulmonary, Critical Care and Sleep Medicine, University of California, Davis, School of Medicine and VA Northern California Health Care System, Sacramento, CA 95817, USA.Division of Pulmonary, Critical Care and Sleep Medicine, University of California, Davis, School of Medicine and VA Northern California Health Care System, Sacramento, CA 95817, USA.Division of Pulmonary, Critical Care and Sleep Medicine, University of California, Davis, School of Medicine and VA Northern California Health Care System, Sacramento, CA 95817, USA.Division of Pulmonary, Critical Care and Sleep Medicine, University of California, Davis, School of Medicine and VA Northern California Health Care System, Sacramento, CA 95817, USA.Division of Pulmonary, Critical Care and Sleep Medicine, University of California, Davis, School of Medicine and VA Northern California Health Care System, Sacramento, CA 95817, USA.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

25922733

Citation

Juarez, Maya M., et al. "Acute Exacerbation of Idiopathic Pulmonary Fibrosis-a Review of Current and Novel Pharmacotherapies." Journal of Thoracic Disease, vol. 7, no. 3, 2015, pp. 499-519.
Juarez MM, Chan AL, Norris AG, et al. Acute exacerbation of idiopathic pulmonary fibrosis-a review of current and novel pharmacotherapies. J Thorac Dis. 2015;7(3):499-519.
Juarez, M. M., Chan, A. L., Norris, A. G., Morrissey, B. M., & Albertson, T. E. (2015). Acute exacerbation of idiopathic pulmonary fibrosis-a review of current and novel pharmacotherapies. Journal of Thoracic Disease, 7(3), pp. 499-519. doi:10.3978/j.issn.2072-1439.2015.01.17.
Juarez MM, et al. Acute Exacerbation of Idiopathic Pulmonary Fibrosis-a Review of Current and Novel Pharmacotherapies. J Thorac Dis. 2015;7(3):499-519. PubMed PMID: 25922733.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Acute exacerbation of idiopathic pulmonary fibrosis-a review of current and novel pharmacotherapies. AU - Juarez,Maya M, AU - Chan,Andrew L, AU - Norris,Andrew G, AU - Morrissey,Brian M, AU - Albertson,Timothy E, PY - 2014/10/09/received PY - 2014/11/26/accepted PY - 2015/4/30/entrez PY - 2015/4/30/pubmed PY - 2015/4/30/medline KW - Idiopathic pulmonary fibrosis (IPF) KW - acute exacerbation (AE) KW - clinical trials KW - drug therapy KW - treatment SP - 499 EP - 519 JF - Journal of thoracic disease JO - J Thorac Dis VL - 7 IS - 3 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. SN - 2072-1439 UR - https://www.unboundmedicine.com/medline/citation/25922733/Acute_exacerbation_of_idiopathic_pulmonary_fibrosis_a_review_of_current_and_novel_pharmacotherapies_ L2 - https://doi.org/10.3978/j.issn.2072-1439.2015.01.17 DB - PRIME DP - Unbound Medicine ER -