A review of current and novel therapies for idiopathic pulmonary fibrosis

Rokhsara Rafii, Maya M. Juarez, Timothy E Albertson, Andrew Chan

Research output: Contribution to journalArticle

105 Scopus citations

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressively fibrotic interstitial lung disease that is associated with a median survival of 2-3 years from initial diagnosis. To date, there is no treatment approved for IPF in the United States, and only one pharmacological agent has been approved outside of the United States. Nevertheless, research over the past 10 years has provided us with a wealth of information on its histopathology, diagnostic work-up, and a greater understanding of its pathophysiology. Specifically, IPF is no longer thought to be a predominantly pro-inflammatory disorder. Rather, the fibrosis in IPF is increasingly understood to be the result of a fibroproliferative and aberrant wound healing cascade. The development of therapeutic targets has shifted in accord with this paradigm change. This review highlights the current understanding of IPF, and the recent as well as novel therapeutics being explored in clinical trials for the treatment of this devastating disease.

Original languageEnglish (US)
Pages (from-to)48-73
Number of pages26
JournalJournal of Thoracic Disease
Volume5
Issue number1
DOIs
StatePublished - Feb 2013

Keywords

  • Clinical trials
  • Idiopathic pulmonary fibrosis/drug therapy
  • Idiopathic pulmonary fibrosis/pathology
  • Molecular targeted therapy

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

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