Acute exacerbation of idiopathic pulmonary fibrosis-A review of current and novel pharmacotherapies

Maya M. Juarez, Andrew Chan, Andrew G. Norris, Brian M Morrissey, Timothy E Albertson

Research output: Contribution to journalArticlepeer-review

57 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)499-519
Number of pages21
JournalJournal of Thoracic Disease
Issue number3
StatePublished - 2015


  • Acute exacerbation (AE)
  • Clinical trials
  • Drug therapy
  • Idiopathic pulmonary fibrosis (IPF)
  • Treatment

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


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