Acute exacerbations of idiopathic pulmonary fibrosis

Harold R. Collard, Bethany B. Moore, Kevin R. Flaherty, Kevin K. Brown, Robert J. Kaner, Talmadge E. King, Joseph A. Lasky, James E. Loyd, Imre Noth, Mitchell A. Olman, Ganesh Raghu, Jesse Roman, Jay H. Ryu, David A. Zisman, Gary W. Hunninghake, Thomas V. Colby, Jim J. Egan, David M. Hansell, Takeshi Johkoh, Naftali KaminskiSoon Kim Dong, Yasuhiro Kondoh, David A. Lynch, Joachim Müller-Quernheim, Jeffrey L. Myers, Andrew G. Nicholson, Moisés Selman, Galen B. Toews, Athol U. Wells, Fernando J. Martinez

Research output: Contribution to journalReview articlepeer-review

826 Scopus citations


The natural history of idiopathic pulmonary fibrosis (IPF) has been characterized as a steady, predictable decline in lung function over time. Recent evidence suggests that some patients may experience a more precipitous course, with periods of relative stability followed by acute deteriorations in respiratory status. Many of these acute deteriorations are of unknown etiology and have been termed acute exacerbations of IPF. This perspective is the result of an international effort to summarize the current state of knowledge regarding acute exacerbations of IPF. Acute exacerbations of IPF aredefinedas acute, clinically significant deteriorations of unidentifiable cause in patients with underlying IPF. Proposed diagnostic criteria include subjective worsening over 30 days or less, new bilateral radiographic opacities, and the absence of infection or another identifiable etiology.The potential pathobiological roles of infection, disordered cell biology, coagulation, and genetics are discussed, and future research directions are proposed.

Original languageEnglish (US)
Pages (from-to)636-643
Number of pages8
JournalAmerican journal of respiratory and critical care medicine
Issue number7
StatePublished - Oct 1 2007


  • Acute exacerbation
  • Definition
  • Diagnosis
  • Pulmonary fibrosis

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine


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